Rare Cancer News & Clinical Trials » Adrenal Gland Cancer

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Adrenal Gland Cancer (252 unread)

Trial - Adrenal Cancer

• 

  Human Placental-Derived Stem Cell Transplantation

  Posted: April 25th, 2012, 8:00am MDT

  Conditions:   Mucopolysaccharidosis I;   Mucopolysaccharidosis VI;   Adrenoleukodystrophy;   Niemann-Pick Disease;   Metachromatic Leukodystrophy;   Wolman Disease;   Krabbe's Disease;   Gaucher's Disease;   Fucosidosis;   Batten Disease;   Severe Aplastic Anemia;   Diamond-Blackfan Anemia;   Amegakaryocytic Thrombocytopenia;   Myelodysplastic Syndrome;   Acute Myelogenous Leukemia;   Acute Lymphocytic Leukemia
  Intervention:   Drug: Human Placental Derived Stem Cell
  Sponsors:   New York Medical College;   New York Medical College
  Not yet recruiting - verified April 2012
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  An Open-label, Multi-center, Expanded Access Study of Pasireotide s.c. in Patients With Cushing's Disease

  Posted: April 12th, 2012, 8:00am MDT

  Condition:   Cushing's Disease
  Intervention:   Drug: Pasireotide sub-cutaneous formulation
  Sponsors:   Novartis;   Novartis Pharmaceuticals
  Available - verified April 2012
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  Does Serum-DXM Increase Diagnostic Accuracy of the Overnight DXM Suppression Test in the Work-up of Cushing's Syndrome?

  Posted: November 15th, 2011, 8:00am MST

  Conditions:   Cushing's Syndrome;   Adrenal Incidentalomas;   Alcoholism;   Obesity
  Intervention:  
  Sponsors:   Haukeland University Hospital;   Haukeland University Hospital
  Recruiting - verified May 2012
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  Study Evaluating The Safety And Efficacy Of Varenicline and Bupropion For Smoking Cessation In Subjects With And Without A History Of Psychiatric Disorders

  Posted: October 14th, 2011, 8:00am MDT

  Condition:   Smoking Cessation
  Interventions:   Drug: Placebo;   Drug: varenicline tartrate;   Drug: bupropion hydrochloride;   Drug: Nicotine Replacement Therapy Patch
  Sponsors:   Pfizer;   Pfizer;   GlaxoSmithKline
  Recruiting - verified May 2012
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  Cediranib and Olaparib in Combination for Recurrent Ovarian or Triple-Negative Breast Cancer

  Posted: April 29th, 2010, 8:00am MDT

  Conditions:   Ovarian Cancer;   Primary Peritoneal Cancer;   Fallopian Tube Cancer
  Interventions:   Drug: cediranib;   Drug: olaparib
  Sponsors:   Dana-Farber Cancer Institute;   Dana-Farber Cancer Institute;   Massachusetts General Hospital;   Beth Israel Deaconess Medical Center;   University of Chicago;   National Cancer Institute (NCI)
  Recruiting - verified April 2012
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  Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors

  Posted: February 25th, 2010, 8:00am MST

  Conditions:   Brain and Central Nervous System Tumors;   Extra-adrenal Paraganglioma
  Interventions:   Drug: temozolomide;   Drug: vorinostat;   Genetic: DNA methylation analysis;   Genetic: western blotting;   Other: laboratory biomarker analysis;   Other: pharmacological study
  Sponsors:   National Cancer Institute (NCI);   Children's Oncology Group;   National Cancer Institute (NCI)
  Active, not recruiting - verified November 2010
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  Seneca Valley Virus-001 and Cyclophosphamide in Treating Young Patients With Relapsed or Refractory Neuroblastoma, Rhabdomyosarcoma, or Rare Tumors With Neuroendocrine Features

  Posted: January 13th, 2010, 8:00am MST

  Conditions:   Adrenocortical Carcinoma;   Gastrointestinal Carcinoid Tumor;   Kidney Cancer;   Neuroblastoma;   Retinoblastoma;   Sarcoma
  Interventions:   Biological: Seneca Valley virus-001;   Drug: cyclophosphamide;   Other: laboratory biomarker analysis;   Other: pharmacological study
  Sponsors:   National Cancer Institute (NCI);   Children's Oncology Group;   National Cancer Institute (NCI)
  Recruiting - verified May 2012
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  Treating Patients With Prostate Cancer That Did Not Respond to Hormone Therapy

  Posted: April 11th, 2007, 8:00am MDT

  Condition:   Prostate Cancer
  Interventions:   Drug: ketoconazole;   Drug: lenalidomide;   Drug: therapeutic hydrocortisone
  Sponsors:   Case Comprehensive Cancer Center;   Case Comprehensive Cancer Center;   National Cancer Institute (NCI)
  Active, not recruiting - verified May 2012
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  Bortezomib With or Without Hormone Therapy in Treating Patients With Relapsed Prostate Cancer

  Posted: February 7th, 2005, 8:00am MST

  Condition:   Prostate Cancer
  Interventions:   Drug: bicalutamide;   Drug: bortezomib;   Drug: flutamide;   Drug: goserelin acetate;   Drug: leuprolide acetate
  Sponsors:   Medical University of South Carolina;   Medical University of South Carolina
  Completed - verified April 2012
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  Docetaxel, Radiation Therapy, and Hormone Therapy in Treating Patients With Locally Advanced Prostate Cancer

  Posted: December 8th, 2004, 8:00am MST

  Condition:   Prostate Cancer
  Interventions:   Drug: bicalutamide;   Drug: docetaxel;   Drug: goserelin acetate;   Drug: leuprolide acetate;   Procedure: adjuvant therapy;   Procedure: neoadjuvant therapy;   Radiation: radiation therapy
  Sponsors:   Medical University of South Carolina;   Medical University of South Carolina
  Active, not recruiting - verified April 2012

Google - Adrenal Cancer

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  3-Year-Old Cancer Free on Clinical Trial - WSET

  Posted: May 24th, 2012, 11:13am MDT

  3-Year-Old Cancer Free on Clinical Trial WSET "She had a large tumor, one growing out of her right adrenal gland, one growing out of her left adrenal gland," Emily explained. "They met in her abdomen and she had spots going up her spine." "That tumor was closing off liver, her stomach and all that ...

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  'New Drug To Fight Against Localized High-Risk Prostate Tumors' - Medical News Today

  Posted: May 23rd, 2012, 1:07pm MDT

  'New Drug To Fight Against Localized High-Risk Prostate Tumors' Medical News Today The male hormone androgen powers prostate cancer growth, and although standard treatments target the production of this hormone in the adrenal glands and testes, they do not target androgen within the tumor itself. However, abiraterone acetate has the ... and more »

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  Hawkathon honours two teenagers lost to cancer - Surrey Now

  Posted: May 23rd, 2012, 11:32pm MDT

  Hawkathon honours two teenagers lost to cancer Surrey Now Both McNeil and Beauchamp were well known in the community for their achievements and their battles with cancer. McNeil, a Seaquam alumni, passed away in January 2011 after a four-year fight with adrenal cancer. She touched many lives with her song, ...

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  Steps toward a cancer cure - Fort Wayne Journal Gazette

  Posted: May 22nd, 2012, 11:22pm MDT

  Steps toward a cancer cure Fort Wayne Journal Gazette Neuroblastoma causes rapidly spreading cancer cells to arise in the adrenal gland, neck, chest or spinal cord. The disease is found in about 500 children a year and has about a 50 percent survival rate, according to the National Institutes of Health.

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  Brandon's Blog: Community Service Equals Leader's and Best - MGoBlue

  Posted: May 21st, 2012, 8:10am MDT

  MGoBlue

  Brandon's Blog: Community Service Equals Leader's and Best MGoBlue The event was to benefit funding of the Millie Schembechler Adrenal Cancer Research Fund. Bo's first wife Millie died in 1992 of adrenal cancer, a very rare form of cancer that strikes about 200 Americans each year. Schembechler and Wikel ran this ... and more »

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  Experimental Drug Helps Fight Some Childhood Cancers, Study Finds - Sudan Vision

  Posted: May 20th, 2012, 12:27pm MDT

  Experimental Drug Helps Fight Some Childhood Cancers, Study Finds Sudan Vision The three types of pediatric cancers currently targeted for this drug therapy include anaplastic large cell lymphoma (ALCL), a type of blood cancer; neuroblastoma, a nerve cancer that usually affects the adrenal gland or the chest; and inflammatory ... and more »

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  Golf tournament raises thousands for girl with cancer - Northwest Cable News

  Posted: May 19th, 2012, 3:01pm MDT

  Golf tournament raises thousands for girl with cancer Northwest Cable News Kalin and Melissa King were crushed when they heard their 2-year-old daughter, Lilyan, had cancer. "She has stage four neuroblastoma," said Melissa. "So she had a tumor on her left adrenal gland, which has now been removed. and more »

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  Golf tournament raises thousands for girl with cancer - KTVB

  Posted: May 19th, 2012, 2:58pm MDT

  Golf tournament raises thousands for girl with cancer KTVB Kalin and Melissa King were crushed when they heard their 2-year-old daughter, Lilyan, had cancer. "She has stage four neuroblastoma," said Melissa. "So she had a tumor on her left adrenal gland, which has now been removed. and more »

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  Ipsilateral Adrenalectomy During RN Hikes Death Risk - Renal and Urology News

  Posted: May 19th, 2012, 2:25pm MDT

  Ipsilateral Adrenalectomy During RN Hikes Death Risk Renal and Urology News Adrenal gland removal was associated with worse overall survival, with a 10-year mortality rate of 26%. In multivariate analysis, ipsilateral adrenalectomy was associated with a 23% increased risk of death. It was not associated with cancer-specific ... and more »

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  Adrenalectomy Plus Nephrectomy Linked to Reduced Survival - Medscape

  Posted: May 19th, 2012, 9:04am MDT

  Adrenalectomy Plus Nephrectomy Linked to Reduced Survival Medscape May 19, 2012 (Atlanta, Georgia) — Overall survival is lower in patients with renal cell carcinoma who underwent radical nephrectomy with concurrent ipsilateral adrenalectomy than in patients with the ipsilateral adrenal gland left intact, according to ... and more »

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  Hormone-depleting drug shows promise against localized high-risk prostate tumors - HealthCanal.com

  Posted: May 18th, 2012, 4:50pm MDT

  Hormone-depleting drug shows promise against localized high-risk prostate tumors HealthCanal.com Androgen, the male hormone, provides the fuel for prostate cancer growth. Conventional therapies target androgen production in the testes and adrenal glands, but not within the tumor itself. Abiraterone acetate is capable of blocking androgen ... and more »

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  Is Kidney Cancer Rare? - dailyRx

  Posted: May 17th, 2012, 2:48pm MDT

  Is Kidney Cancer Rare? dailyRx The average patient was a 62 year old male, and the cancer was usually discovered by accident. The tumors were normally about four centimeters in diameter. Most patients were treated by removing kidney, lymph, adrenal glands, and surrounding fat.

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  Why do I Relay for Life? - Moberly Monitor Index

  Posted: May 17th, 2012, 12:13pm MDT

  Why do I Relay for Life? Moberly Monitor Index He said, “ we took it all out, we got the whole tumor. “ The doctor had removed the tumor, some blood vessels from the kidney, some lymph nodes and an adrenal gland. Everything was sent to pathology for testing and we waited again. and more »

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  Experimental Drug Helps Fight Some Childhood Cancers, Study Finds - Doctors Lounge

  Posted: May 16th, 2012, 8:21pm MDT

  Experimental Drug Helps Fight Some Childhood Cancers, Study Finds Doctors Lounge The three types of pediatric cancers currently targeted for this drug therapy include anaplastic large cell lymphoma (ALCL), a type of blood cancer; neuroblastoma, a nerve cancer that usually affects the adrenal gland or the chest; and inflammatory ... and more »

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  Hockey players on ice for 11 days in fundraiser for cancer - Ottawa Citizen

  Posted: May 16th, 2012, 6:15pm MDT

  Hockey players on ice for 11 days in fundraiser for cancer Ottawa Citizen His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Hockey players on ice for 11 days in fundraiser for cancer - Faceoff.com

  Posted: May 16th, 2012, 6:02pm MDT

  Hockey players on ice for 11 days in fundraiser for cancer Faceoff.com His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Hockey players on ice for 11 days best world record in fundraiser for cancer - Hamilton Spectator

  Posted: May 16th, 2012, 5:38pm MDT

  Hockey players on ice for 11 days best world record in fundraiser for cancer Hamilton Spectator Diamond is in remission from a rare form of adrenal cancer. CHESTERMERE, ALTA. Lyall Marshall pushed harder through the pain, the numb toes and utter exhaustion whenever he saw his daughter's dimpled smile through the Plexiglas. and more »

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  World record 11-day hockey game benefits cancer - CBC.ca

  Posted: May 16th, 2012, 4:34pm MDT

  World record 11-day hockey game benefits cancer CBC.ca His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Hormone-depleting drug shows promise against localized high-risk prostate tumors - Science Codex

  Posted: May 16th, 2012, 3:12pm MDT

  Science Codex

  Hormone-depleting drug shows promise against localized high-risk prostate tumors Science Codex Androgen, the male hormone, provides the fuel for prostate cancer growth. Conventional therapies target androgen production in the testes and adrenal glands, but not within the tumor itself. Abiraterone acetate is capable of blocking androgen ... and more »

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  Hockey players on ice for 11 days best world record in fundraiser for cancer - 570 News

  Posted: May 16th, 2012, 3:10pm MDT

  Hockey players on ice for 11 days best world record in fundraiser for cancer 570 News His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Hockey players on ice for 11 days best world record in fundraiser for cancer - News1130

  Posted: May 16th, 2012, 3:03pm MDT

  Hockey players on ice for 11 days best world record in fundraiser for cancer News1130 His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Hockey players on ice for 11 days best world record in fundraiser for cancer - 680 News

  Posted: May 16th, 2012, 2:56pm MDT

  Hockey players on ice for 11 days best world record in fundraiser for cancer 680 News His oldest daughter, six-year-old Diamond, is in remission from a rare form of adrenal cancer. She captured hearts across the country last summer when she darted out to greet the Duchess of Cambridge on a royal tour stop in Calgary. and more »

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  Boy with deadly cancer captures hearts of people in Houston area - KY3

  Posted: May 15th, 2012, 2:17pm MDT

  Boy with deadly cancer captures hearts of people in Houston area KY3 "He had a tumor that grew out of his adrenal gland on top of his kidney, wrapped around his aorta, filled up his abdomen, split his diaphragm and settled on the bottom half of his lungs," Myrick said. Liam is being treated at St. Louis Children's ...

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  New Therapies for Prostate Cancer: The Practicalities - OncLive

  Posted: May 15th, 2012, 1:30pm MDT

  OncLive

  New Therapies for Prostate Cancer: The Practicalities OncLive Abiraterone severely inhibits testosterone production by the adrenal gland and in the tumor itself. However, it also increases mineralocorticoid production, creating the need to administer it together with prednisone to abrogate the clinical effects of ... and more »

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  Safer Kidney Cancer Surgery Under-Used for Poorer, Sicker Medicare & Medicaid ... - Newswise (press release)

  Posted: May 15th, 2012, 11:51am MDT

  Safer Kidney Cancer Surgery Under-Used for Poorer, Sicker Medicare & Medicaid ... Newswise (press release) Standard treatment for small kidney tumors has traditionally been radical nephrectomy (RN) – surgical removal of the entire kidney, part of the ureter, the adrenal gland, and some surrounding tissue. The less-extreme PN became possible with ... and more »

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  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... - Science Codex

  Posted: May 15th, 2012, 11:43am MDT

  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... Science Codex Standard treatment for small kidney tumors has traditionally been radical nephrectomy (RN) – surgical removal of the entire kidney, part of the ureter, the adrenal gland, and some surrounding tissue. The less-extreme PN became possible with ... and more »

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  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... - Medical Xpress

  Posted: May 15th, 2012, 10:59am MDT

  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... Medical Xpress Standard treatment for small kidney tumors has traditionally been radical nephrectomy (RN) – surgical removal of the entire kidney, part of the ureter, the adrenal gland, and some surrounding tissue. The less-extreme PN became possible with ... and more »

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  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... - EurekAlert (press release)

  Posted: May 15th, 2012, 10:35am MDT

  Safer kidney cancer surgery under-used for poorer, sicker Medicare, Medicaid ... EurekAlert (press release) Standard treatment for small kidney tumors has traditionally been radical nephrectomy (RN) – surgical removal of the entire kidney, part of the ureter, the adrenal gland, and some surrounding tissue. The less-extreme PN became possible with ... and more »

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  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... - EON: Enhanced Online News (press release)

  Posted: May 14th, 2012, 8:07pm MDT

  EON: Enhanced Online News (press release)

  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... EON: Enhanced Online News (press release) NEW YORK--(EON: Enhanced Online News)--New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, including tumors of the kidney, lung and hematologic malignancies, at the Annual ... and more »

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  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... - PharmaLive (press release) (subscription)

  Posted: May 14th, 2012, 12:02pm MDT

  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... PharmaLive (press release) (subscription) NEW YORK--(BUSINESS WIRE)--May 14, 2012 - New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, including tumors of the kidney, lung and hematologic malignancies, ... and more »

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  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... - PharmaLive.com (press release)

  Posted: May 14th, 2012, 11:55am MDT

  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... PharmaLive.com (press release) NEW YORK--(BUSINESS WIRE)--May 14, 2012 - New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, including tumors of the kidney, lung and hematologic malignancies, ... and more »

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  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... - PharmiWeb.com (press release)

  Posted: May 14th, 2012, 5:12am MDT

  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... PharmiWeb.com (press release) New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, including tumors of the kidney, lung and hematologic malignancies, at the Annual Meeting of the American Society of ... and more »

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  PFIZER INC. : Pfizer To Present New Data In Advanced Kidney, Lung And ... - 4-traders

  Posted: May 14th, 2012, 4:06am MDT

  PFIZER INC. : Pfizer To Present New Data In Advanced Kidney, Lung And ... 4-traders Pfizer Inc. : Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At 2012 ASCO Annual Meeting New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, ... and more »

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  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... - MarketWatch (press release)

  Posted: May 14th, 2012, 4:02am MDT

  Pfizer To Present New Data In Advanced Kidney, Lung And Hematologic Cancers At ... MarketWatch (press release) New data will be presented on targeted cancer treatments from the Pfizer Oncology portfolio across three key therapeutic categories, including tumors of the kidney, lung and hematologic malignancies, at the Annual Meeting of the American Society of ... and more »

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  Fighting for Coach Moose - Cherry Hill Courier Post

  Posted: May 14th, 2012, 11:15pm MDT

  Fighting for Coach Moose Cherry Hill Courier Post But, off the field, the Cougars have all been winners, because the entire team has rallied behind head coach Charlie Musumeci, who is battling adrenal cancer and has had to curtail his coaching schedule so he can be treated. Musumeci, a vivacious man ...

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  Hockey Marathon skates into seventh day - CBC.ca

  Posted: May 12th, 2012, 4:46pm MDT

  Hockey Marathon skates into seventh day CBC.ca The event is looking to fundraise $1.5 million for pediatric cancer research and intensive care equipment for the Alberta Children's Hospital. Lyle Marshall is skating for his six-year-old daughter Diamond, who has been battling a rare form of adrenal ... and more »

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  Diamond's shine an inspiration - Calgary Sun

  Posted: May 5th, 2012, 2:55pm MDT

  Diamond's shine an inspiration Calgary Sun By Katie Schneider ,Calgary Sun More than a year after being diagnosed with a rare form of adrenal cancer, Diamond is home recovering from a bone marrow transplant, almost completely back to being a normal six-year-old girl. Even if normal means having ...

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  Combination chemotherapy shows benefits for adrenal cancer patients - Medical Xpress

  Posted: May 4th, 2012, 12:29am MDT

  Combination chemotherapy shows benefits for adrenal cancer patients Medical Xpress Endocrinologists at the University of Birmingham have played a key role in a major international clinical trial which has found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up ... and more »

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  Combination chemotherapy shows benefits for adrenal cancer patients - HealthCanal.com

  Posted: May 3rd, 2012, 1:25pm MDT

  Combination chemotherapy shows benefits for adrenal cancer patients HealthCanal.com Endocrinologists at the University of Birmingham have played a key role in a major international clinical trial which has found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up ... and more »

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  Research and Markets: Adrenocortical Carcinoma (Adrenal Cortex Cancer ... - SunHerald.com

  Posted: May 3rd, 2012, 12:40pm MDT

  Research and Markets: Adrenocortical Carcinoma (Adrenal Cortex Cancer ... SunHerald.com DUBLIN -- Research and Markets [www.researchandmarkets.com] has announced the addition of Global Markets Direct's new report "Adrenocortical Carcinoma (Adrenal Cortex Cancer) - Pipeline Review, ... and more »

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  Combination chemo holds back cancer - Public Service

  Posted: May 3rd, 2012, 2:57am MDT

  Combination chemo holds back cancer Public Service Endocrinologists at the University of Birmingham have found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up to a fifth longer. According to the New England Journal of Medicine, ... and more »

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  Toddler battling against cancer - Sunderland Echo

  Posted: May 3rd, 2012, 12:25am MDT

  Sunderland Echo

  Toddler battling against cancer Sunderland Echo Just a week after developing symptoms, the Castletown youngster was diagnosed with the rapidly spreading disease in his stomach, hip, adrenal gland and lymph nodes. Mum Jemma, 28, who has had a tattoo in honour of her “hero”, said: “He had always been ...

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  Mixed Results for New Tx in Adrenal Cancer - MedPage Today

  Posted: May 2nd, 2012, 4:05pm MDT

  Mixed Results for New Tx in Adrenal Cancer MedPage Today Patients with advanced adrenocortical carcinoma lived no longer when standard therapy was combined with three-agent chemotherapy but had a significantly higher response rate and progression-free survival (PFS). Note that the three-drug combination did ... and more »

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  Forest Hills Woman Never Gives Up in Cancer Battle - Patch.com

  Posted: May 2nd, 2012, 2:05am MDT

  Forest Hills Woman Never Gives Up in Cancer Battle Patch.com In 2011, she was again diagnosed, this time with stage four breast cancer that spread to the lung, lymph nodes, adrenal glands and bones in the leg. She's undergoing surgery, radiation and chemotherapy in her current battle. The journey has been filled ... and more »

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  Terminally-Ill Belly Dancer Takes Control of Her Destiny - Patch.com

  Posted: April 30th, 2012, 3:09pm MDT

  Terminally-Ill Belly Dancer Takes Control of Her Destiny Patch.com By Zak Koeske Rather than give up after learning last August that her adrenal cancer had returned and that she likely had only a few months to live, Sigi Nissimov used the opportunity to regain control of her life. With radiation out of the question ...

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  Doctor recounts his cancer fight in poetry - Inverness Courier

  Posted: April 27th, 2012, 11:34pm MDT

  Inverness Courier

  Doctor recounts his cancer fight in poetry Inverness Courier But four years ago the roles were reversed when he learned he had an extensive and extremely rare form of adrenal cancer — so rare that he had to travel to London for radical, life-saving surgery. Now, just two months after completing a course of ...

• 

  Study: Anxiety Increases Cancer Severity in Mice - Fars News Agency

  Posted: April 26th, 2012, 11:40pm MDT

  Fars News Agency

  Study: Anxiety Increases Cancer Severity in Mice Fars News Agency The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system -- the "fight or flight" ... and more »

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  Anxiety increases cancer severity in mice, study shows - Science Daily (press release)

  Posted: April 25th, 2012, 4:12pm MDT

  Anxiety increases cancer severity in mice, study shows Science Daily (press release) The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system -- the "fight or flight" ... and more »

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  Anxiety increases cancer severity in mice, Stanford study shows - Science Codex

  Posted: April 25th, 2012, 2:05pm MDT

  Anxiety increases cancer severity in mice, Stanford study shows Science Codex The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system — the "fight or flight" ... and more »

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  Anxiety increases cancer severity in mice, study shows - HealthCanal.com

  Posted: April 25th, 2012, 1:43pm MDT

  Anxiety increases cancer severity in mice, study shows HealthCanal.com The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system — the “fight or flight” ... and more »

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  Anxiety increases cancer severity in mice, Stanford study shows - EurekAlert (press release)

  Posted: April 25th, 2012, 1:15pm MDT

  Anxiety increases cancer severity in mice, Stanford study shows EurekAlert (press release) The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system — the "fight or flight" ... and more »

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  Anxiety increases cancer severity in mice, research finds - Medical Xpress

  Posted: April 25th, 2012, 1:11pm MDT

  Anxiety increases cancer severity in mice, research finds Medical Xpress The high-strung mice were also making fewer of the chemical signals that fire up an immune attack on the tumors. Lastly, the researchers looked at the hormone corticosterone. In mice and other animals, the adrenal system — the "fight or flight" ... and more »

• 

  Star Wars Characters Come To Wausau To Help 9 Year Old Boy's Benefit - WSAW

  Posted: April 22nd, 2012, 4:22pm MDT

  Star Wars Characters Come To Wausau To Help 9 Year Old Boy's Benefit WSAW For nearly two years, nice year old Kyle Pagel battled a rare type of cancer called neuroblastoma. It's usually found in infants and younger children. As a result, Kyle had to have an adrenal gland removed, as well as two stem cell transplants. and more »

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  Riley cancer patients dress up for hospital's prom - Indianapolis Star

  Posted: April 22nd, 2012, 10:06pm MDT

  Riley cancer patients dress up for hospital's prom Indianapolis Star Jessica, who just finished chemotherapy to treat an adrenal carcinoma, was looking for something in black. But after the two sisters disappeared into a makeshift dressing room, Jessica glided out in a form-fitting orange bejeweled number.

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  Brave Thomas Cammack loses cancer battle after Gruffalo wish comes true - This is Hull and East Riding

  Posted: April 20th, 2012, 11:18pm MDT

  Brave Thomas Cammack loses cancer battle after Gruffalo wish comes true This is Hull and East Riding Thomas was diagnosed last year with neuroblastoma, a cancer which spread from his adrenal gland to his skull, bone marrow, blood and pelvis. Despite completing initial treatment, two days before his fourth birthday, the family were told routine ... and more »

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  Thomas Cammack loses his battle with cancer - The Press, York

  Posted: April 19th, 2012, 1:14am MDT

  Thomas Cammack loses his battle with cancer The Press, York Thomas was diagnosed last year with neuroblastoma – a cancer which spread from his adrenal gland to his skull, bone marrow, blood and pelvis – and two weeks ago, underwent surgery to remove a tumour from his brain. He appeared to be recovering well, ... and more »

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  Lessons from Cancer: What happens when cancer keeps growing? - Wicked Local

  Posted: April 18th, 2012, 8:25pm MDT

  Lessons from Cancer: What happens when cancer keeps growing? Wicked Local By Joyce Rothman My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right ...

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Leavenworth Times (blog)

  Posted: April 16th, 2012, 5:08pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Leavenworth Times (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Heber Springs Sun-Times (blog)

  Posted: April 16th, 2012, 4:49pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Heber Springs Sun-Times (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Nebraska City News Press (blog)

  Posted: April 16th, 2012, 4:25pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Nebraska City News Press (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Oak Ridger (blog)

  Posted: April 16th, 2012, 4:24pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Oak Ridger (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Waynesboro Record Herald (blog)

  Posted: April 16th, 2012, 4:24pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Waynesboro Record Herald (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Utica Observer Dispatch (blog)

  Posted: April 16th, 2012, 4:24pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Utica Observer Dispatch (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - New Philadelphia Times Reporter (blog)

  Posted: April 16th, 2012, 4:19pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer New Philadelphia Times Reporter (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Saugerties Post Star (blog)

  Posted: April 16th, 2012, 4:17pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Saugerties Post Star (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Canton Daily Ledger (blog)

  Posted: April 16th, 2012, 4:15pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Canton Daily Ledger (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? ~ Lessons from Cancer - Pekin Daily Times (blog)

  Posted: April 16th, 2012, 4:13pm MDT

  What happens when cancer keeps growing? ~ Lessons from Cancer Pekin Daily Times (blog) By Joyce My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right upper lung ... and more »

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  What happens when cancer keeps growing? - Fall River Herald News

  Posted: April 13th, 2012, 8:24am MDT

  What happens when cancer keeps growing? Fall River Herald News By Joyce Rothman My head hurts under the emotional weight of these cancers growing in my lungs, lymph nodes, adrenal gland and pelvic bone. And now there is the question of a liver lesion being malignant. Plus, there's a new finding that the right ... and more »

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  Neuroblastoma is an aggressive type of childhood cancer - This is Hull and East Riding

  Posted: April 13th, 2012, 6:36am MDT

  Neuroblastoma is an aggressive type of childhood cancer This is Hull and East Riding It is a cancer of the nervous system and can therefore affect every part of the body. Neuroblastoma usually starts in the adrenal glands, but it is also quite common for it to start in the neck or chest area. The disease spreads rapidly to far-reaching ... and more »

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  Scavenger hunt to aid family: 9-year-old's cancer diagnosis leads to mountain ... - Wausau Daily Herald

  Posted: April 13th, 2012, 1:41am MDT

  Scavenger hunt to aid family: 9-year-old's cancer diagnosis leads to mountain ... Wausau Daily Herald The 9-year-old boy was found to have neuroblastoma, a rare childhood cancer, in September 2010. He has had five rounds of chemotherapy, surgery to remove a tumor on his left adrenal gland, five months of immunotherapy treatments and two back-to-back ... and more »

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  UPDATE: Data Published in Premier Endocrinology Journal Shows Korlym(TM ... - Healthcare Global (press release)

  Posted: April 11th, 2012, 2:38pm MDT

  UPDATE: Data Published in Premier Endocrinology Journal Shows Korlym(TM ... Healthcare Global (press release) Forty-three had Cushing's disease (an ACTH-producing tumor of the pituitary gland), of which 42 had prior surgery, four patients had ectopic ACTH-producing tumors and three had adrenal cancer. Of the 29 patients enrolled in the study who were glucose ... and more »

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  Holland family looks for second opinion in cancer fight - HollandSentinel.com

  Posted: April 7th, 2012, 11:23am MDT

  Holland family looks for second opinion in cancer fight HollandSentinel.com By PEG MCNICHOL For two weekends, Jesenia Lazaro and friends have sold donated items via garage sales, hoping to raise at least $3000 to help her son, Jesus Arce, 7, who was diagnosed with adrenal cancer last June. Lazaro wants Jesus to see doctors at ...

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  Newburgh mother speaks out over believed cancer-research scam - 14 News WFIE Evansville

  Posted: April 6th, 2012, 2:21pm MDT

  Newburgh mother speaks out over believed cancer-research scam 14 News WFIE Evansville Wade was seen by several doctors, who confirmed he had neuro-blastoma, a rare solid tumor on his kidneys. "It started on the adrenal gland, it invaded his kidney and it metastasized into his bone marrow," Debruin said. Although the survival rates are ...

• 

  Neuroendocrine Tumor Program Recognized for Excellence - Newswise (press release)

  Posted: April 6th, 2012, 11:49am MDT

  Neuroendocrine Tumor Program Recognized for Excellence Newswise (press release) Under Dr. Libutti's direction, a multi-disciplinary team of specialists evaluates and treats all types and stages of endocrine and neuroendocrine tumors, both benign and malignant. These include tumors associated with the thyroid, parathyroid, adrenal ...

• 

  Top Doctors in Chicago: Surgery - Chicagomag.com

  Posted: April 4th, 2012, 10:34am MDT

  Top Doctors in Chicago: Surgery Chicagomag.com 773-564-6120 MICHAEL ABECASSIS Kidney and liver transplant; pancreatic and biliary surgery; liver cancer. NORTHWESTERN. 312-695-8900 PETER ANGELOS Thyroid and parathyroid cancer and surgery; pheochromocytoma; adrenal tumors; ethics. CHICAGO. and more »

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  Baseline Hormone Levels Appear to Predict Survival in Metastatic, Castration ... - Newswise (press release)

  Posted: April 3rd, 2012, 1:48pm MDT

  Baseline Hormone Levels Appear to Predict Survival in Metastatic, Castration ... Newswise (press release) If confirmed, these data mean that levels of hormones, specifically adrenal androgens, may provide physicians with another way to predict the efficacy of therapy in patients with metastatic, castration-resistant prostate cancer, according to Charles J. and more »

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  Baseline hormone levels may predict survival in metastatic, castration ... - Medical Xpress

  Posted: April 3rd, 2012, 1:44pm MDT

  Baseline hormone levels may predict survival in metastatic, castration ... Medical Xpress If confirmed, these data mean that levels of hormones, specifically adrenal androgens, may provide physicians with another way to predict the efficacy of therapy in patients with metastatic, castration-resistant prostate cancer, according to Charles J. and more »

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  Baseline hormone levels may predict survival in metastatic, castration ... - Science Codex

  Posted: April 3rd, 2012, 1:36pm MDT

  Baseline hormone levels may predict survival in metastatic, castration ... Science Codex If confirmed, these data mean that levels of hormones, specifically adrenal androgens, may provide physicians with another way to predict the efficacy of therapy in patients with metastatic, castration-resistant prostate cancer, according to Charles J. and more »

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  Baseline hormone levels may predict survival in metastatic, castration ... - EurekAlert (press release)

  Posted: April 3rd, 2012, 1:29pm MDT

  Baseline hormone levels may predict survival in metastatic, castration ... EurekAlert (press release) If confirmed, these data mean that levels of hormones, specifically adrenal androgens, may provide physicians with another way to predict the efficacy of therapy in patients with metastatic, castration-resistant prostate cancer, according to Charles J. and more »

• 

  3000-volt jab that shrunk my 'inoperable' cancer by half - Daily Mail

  Posted: April 2nd, 2012, 4:29pm MDT

  Daily Mail

  3000-volt jab that shrunk my 'inoperable' cancer by half Daily Mail The cancer was spreading, and the surgeons felt cutting it out wouldn't stop it, so decided to do nothing. It was devastating and I was really frightened. I'd been diagnosed with adrenal cortical cancer — cancer of the adrenal glands, which sit next to ...

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  Even while battling cancer, Pastor Leah stayed committed to family, her church - Winona Daily News

  Posted: March 31st, 2012, 12:10am MDT

  Even while battling cancer, Pastor Leah stayed committed to family, her church Winona Daily News A Labor Day surgery was scheduled to remove the golf ball-sized growth. It didn't remove the cancer. Around Christmas 2010, tests showed a recurrence of stage four metastatic cancer. It had spread to her adrenal gland, left lung and right hip.

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  Kidney cancer subtype study finds low recurrence and cancer death rates - AnaesthesiaUK

  Posted: March 30th, 2012, 12:05am MDT

  Kidney cancer subtype study finds low recurrence and cancer death rates AnaesthesiaUK Most patients (62%) had radical nephrectomy surgery, where the entire kidney and its collecting system is removed, along with the adrenal gland, the fat tissue around the kidney and the associated lymph glands. At follow-up, 81% of the patients were ... and more »

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  Kidney cancer subtype study finds low recurrence and cancer death rates - Medical Xpress

  Posted: March 29th, 2012, 6:42am MDT

  Kidney cancer subtype study finds low recurrence and cancer death rates Medical Xpress Most patients (62%) had radical nephrectomy surgery, where the entire kidney and its collecting system is removed, along with the adrenal gland, the fat tissue around the kidney and the associated lymph glands. At follow-up, 81% of the patients were ... and more »

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  Kidney cancer subtype study finds low recurrence and cancer death rates - EurekAlert (press release)

  Posted: March 29th, 2012, 6:10am MDT

  Kidney cancer subtype study finds low recurrence and cancer death rates EurekAlert (press release) Most patients (62%) had radical nephrectomy surgery, where the entire kidney and its collecting system is removed, along with the adrenal gland, the fat tissue around the kidney and the associated lymph glands. At follow-up, 81% of the patients were ... and more »

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  The Magen Boys Plan to “Wrestle For A Cure” - Shalom Life

  Posted: March 28th, 2012, 2:33pm MDT

  The Magen Boys Plan to “Wrestle For A Cure” Shalom Life On April 16 th , twin brothers Jian and Page Magen, will be hosting 'Wrestle For A Cure', an “over the top, family friendly, professional wrestling show dedicated to educating and supporting Adrenal Gland Cancer Research at The Princess Margaret ...

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  The R337H mutation in TP53 and breast cancer in Brazil - 7thSpace Interactive (press release)

  Posted: March 28th, 2012, 4:44am MDT

  The R337H mutation in TP53 and breast cancer in Brazil 7thSpace Interactive (press release) Germline mutations in p53 are associated with the Li-Fraumeni Syndrome which is characterized by childhood cancers, including pediatric adrenal cortical carcinomas and early onset breast cancer. The high incidence of adrenal cortical carcinomas in ...

• 

  Avoid Stress? Adrenal Fatigue & Outsmarting Stress: Teleseminar - NewsReleaseWire.com (press release)

  Posted: March 27th, 2012, 8:35am MDT

  Avoid Stress? Adrenal Fatigue & Outsmarting Stress: Teleseminar NewsReleaseWire.com (press release) This Thursday, March 29th, 2012 at 8 PM eastern (www.time.gov), I have the pleasure of discussing the "Adrenal Fatigue & Outsmarting Stress" with Dr. Lena Edwards. "Avoid Stress". You've heard that before. So what are you supposed to do?

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  Hope for Kidney cancer patient challenging insurer's decision - Northwest Cable News

  Posted: March 26th, 2012, 1:34pm MDT

  Hope for Kidney cancer patient challenging insurer's decision Northwest Cable News But in 2007, doctors discovered the tumors had returned. This time, to his left lung, adrenal gland and pancreas. "I said, 'Well, where are the gloves? Give me the gloves, I'll start fighting it again,'" said Gonzales. And when a brain tumor was ... and more »

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  Hope for Kidney cancer patient challenging insurer's decision - KING5.com

  Posted: March 26th, 2012, 1:27pm MDT

  Hope for Kidney cancer patient challenging insurer's decision KING5.com But in 2007, doctors discovered the tumors had returned. This time, to his left lung, adrenal gland and pancreas. "I said, 'Well, where are the gloves? Give me the gloves, I'll start fighting it again,'" said Gonzales. And when a brain tumor was ... and more »

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  Family joining Children's Cancer Run for brave Billingham boy, six - Gazette Live

  Posted: March 24th, 2012, 3:13am MDT

  Family joining Children's Cancer Run for brave Billingham boy, six Gazette Live Lisa and Ryan's dad Tim got the crushing news their son had cancer when he was just three. At the time, the family was living in South Africa where doctors revealed Ryan had a mass down his left side from his adrenal gland to the top of his spine, ...

PubMed - Adrenal Cancer

• 

  Molecular mechanisms of RET receptor-mediated oncogenesis in multiple endocrine neoplasia 2.

  Fetched: May 26th, 2012, 11:00pm MDT

  Molecular mechanisms of RET receptor-mediated oncogenesis in multiple endocrine neoplasia 2.

  Clinics (Sao Paulo). 2012;67 Suppl 1:77-84

  Authors: Wagner SM, Zhu S, Nicolescu AC, Mulligan LM

  Abstract
  Multiple endocrine neoplasia type 2 is an inherited cancer syndrome characterized by tumors of thyroid and adrenal tissues. Germline mutations of the REarranged during Transfection (RET) proto-oncogene, leading to its unregulated activation, are the underlying cause of this disease. Multiple endocrine neoplasia type 2 has been a model in clinical cancer genetics, demonstrating how knowledge of the genetic basis can shape the diagnosis and treatment of the disease. Here, we discuss the nature and effects of the most common recurrent mutations of RET found in multiple endocrine neoplasia type 2. Current understanding of the molecular mechanisms of RET mutations and how they alter the structure and function of the RET protein leading to its aberrant activation, and the effects on RET localization and signaling are described.
  

  PMID: 22584710 [PubMed - in process]

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  Report from the 29th World Congress of Endourology and SWL (November 30-December 3, 2011 - Kyoto, Japan).

  Fetched: May 26th, 2012, 11:00pm MDT

  Report from the 29th World Congress of Endourology and SWL (November 30-December 3, 2011 - Kyoto, Japan).

  Drugs Today (Barc). 2012 Feb;48(2):163-71

  Authors: Rabasseda X

  Abstract
  Kyoto is a city of surprises: from the most beautiful castles and temples known far and wide across the world, to the humblest temples, as beautiful as those appearing in all tourist guides, just hidden away in small lanes among busy traffic of bicycles and pedestrians shopping in markets or attending to their daily business. Add the innumerable tourists busily exploring the city's attractions, although this was not the reason for visiting Kyoto, which was the site of this year's World Congress of Endourology and SWL. Nevertheless, maybe as a compensation, the meeting was held in Kokusaikaikan, literally the International Conference Centre, which is a modern facility in the city outskirts, actually adjoining the Takaragaike park, with a beautiful lake and the mountains, brightly led by the trees' red leaves, just visible through the main hall windows.
  

  PMID: 22384455 [PubMed - indexed for MEDLINE]

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  Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis.

  Fetched: May 26th, 2012, 11:00pm MDT

  Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis.

  Hormones (Athens). 2011 Oct-Dec;10(4):317-25

  Authors: Hagemann K, Zanolari Calderari M, Perren A, Cree I, Mullis PE, Flück CE

  Abstract
  Adrenocortical tumors are rare in children and present with variable signs depending on the type of hormone excess. We herein describe the unusual presentation of a child with adrenocortical tumor and introduce the concept of in vitro chemosensitivity testing. CASE REPORT: A 10.5-year-old girl presented with hypertrichosis/hirsutism and weight loss. The weight loss and behavioral problems, associated with halted puberty and growth, led to the initial diagnosis of anorexia nervosa. However, subsequent weight gain but persisting arrest in growth and puberty and the appearance of central fat distribution prompted further evaluation. RESULTS AND FOLLOW-UP: 24h-urine free cortisol was elevated. Morning plasma ACTH was undetectable, while cortisol was elevated and circadian rhythmicity was absent. Thus a hormonally active adrenal cortical tumor (ACT) was suspected. On magnetic resonance imaging (MRI) a unilateral, encapsulated tumor was found which was subsequently removed surgically. Tissue was investigated histologically and for chemosensitivity in primary cell cultures. Although there were some risk factors for malignancy, the tumor was found to be a typical adenoma. Despite this histology, tumor cells survived in culture and were sensitive to cisplatin in combination with gemcitabine or paclitaxel. At surgery, the patient was started on hydrocortisone replacement which was unsuccessfully tapered over 3 months. Full recovery of the hypothalamus-pituitary-adrenal axis occurred only after 3 years. CONCLUSIONS: The diagnosis of a hormonally active adrenocortical tumor is often delayed because of atypical presentation. Cortisol replacement following unilateral tumor excision is mandatory and may be required for months or years. Individualized chemosensitivity studies carried out on primary cultures established from the tumor tissue itself may provide a tool in evaluating the effectiveness of chemotherapeutic drugs in the event that the adrenocortical tumor may prove to be carcinoma.
  

  PMID: 22281889 [PubMed - indexed for MEDLINE]

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  Progressively increased patterns of subclinical cortisol hypersecretion in adrenal incidentalomas differently predict major metabolic and cardiovascular outcomes: a large cross-sectional study.

  Fetched: May 26th, 2012, 11:00pm MDT

  Progressively increased patterns of subclinical cortisol hypersecretion in adrenal incidentalomas differently predict major metabolic and cardiovascular outcomes: a large cross-sectional study.

  Eur J Endocrinol. 2012 Apr;166(4):669-77

  Authors: Di Dalmazi G, Vicennati V, Rinaldi E, Morselli-Labate AM, Giampalma E, Mosconi C, Pagotto U, Pasquali R

  Abstract
  BACKGROUND: Subclinical Cushing's syndrome (SCS) is defined as alterations in hypothalamic-pituitary-adrenal axis without classic signs/symptoms of glucocorticoid excess. Whether SCS leads to metabolic and cardiovascular diseases is still controversial.
  AIM: To evaluate the prevalence of hypertension, type 2 diabetes (T2D), coronary heart disease (CHD), ischemic stroke, osteoporosis, and fractures, and their relationship to increasing patterns of subclinical hypercortisolism, in patients with nonsecreting adrenal adenomas (NSA) and SCS.
  METHODS: Using the 1 mg dexamethasone suppression test (DST), 348 patients were classified as follows: 203 were defined as NSA and 19 SCS, using the most stringent cutoff values (<50 and >138 nmol/l respectively). Patients with cortisol post-DST (50-138 nmol/l) were considered as intermediate phenotypes and classified as minor (n=71) and major (n=55) using plasma ACTH and/or urinary free cortisol as additional diagnostic tools.
  RESULTS: SCS patients showed higher prevalence of T2D, CHD, osteoporosis, and fractures with respect to NSA. Intermediate phenotypes also showed higher prevalence of CHD and T2D with respect to NSA. The prevalence of all clinical outcomes was not different between intermediate phenotype patients, which were therefore considered as a single group (IP) for multivariate logistic regression analysis: both IP and SCS-secreting patterns showed a significant association with CHD (odds ratio (OR), 4.09; 95% confidence interval (CI), 1.47-11.38 and OR, 6.10; 95% CI, 1.41-26.49 respectively), independently of other potential risk factors. SCS was also independently associated with osteoporosis (OR, 5.94; 95% CI, 1.79-19.68).
  CONCLUSIONS: Patterns of increasing subclinical hypercortisolism in adrenal adenomas are associated with increased prevalence of adverse metabolic and cardiovascular outcomes, independently of other potential risk factors.
  

  PMID: 22267278 [PubMed - indexed for MEDLINE]

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  [Microwave tumor ablation. New devices, new applications?].

  Fetched: May 26th, 2012, 11:00pm MDT

  [Microwave tumor ablation. New devices, new applications?].

  Radiologe. 2012 Jan;52(1):22-8

  Authors: Hoffmann R, Rempp H, Clasen S

  Abstract
  CLINICAL ISSUE: The majority of patients with hepatic malignancies are not amenable to surgical resection. In some of these cases minimally invasive ablative therapies are a treatment option. STANDARD TREATMENT: Besides radiofrequency ablation, the most common ablative therapies are cryoablation, laser ablation and microwave ablation. TREATMENT INNOVATIONS: The classic fields of application of microwave ablation are the treatment of malignancies of the liver, kidneys and lungs. Furthermore, cases of treatment of bone tumors and tumors of the adrenal gland have been reported as well as treatment of secondary hypersplenism. PERFORMANCE: The manufacturers of microwave systems pursue different strategies to reach an optimal ablation zone, such as water or gas cooling of the antenna, the simultaneous use of different antennas or an automatic modulation of the microwave energy and frequency. ACHIEVEMENTS: In contrast to other tumor ablation methods microwave ablation causes a direct heating of a tissue volume, thus this method is less vulnerable to the cooling effect of vessels in the ablation zone. Moreover the electric conductivity of the treated tissue does not influence microwave radiation so that microwave ablation has advantages for the treatment of high-resistance organs, such as the lungs or bone. Some publications have shown that microwave ablation causes larger ablation zones in less time in comparison to radiofrequency ablation. PRACTICAL RECOMMENDATIONS: Classic indications for microwave ablation are the treatment of malignancies of the liver, lungs and kidneys. Initial technical problems have been solved, so that an increasing significance of the microwave ablation among the ablative therapies is to be expected.
  

  PMID: 22249698 [PubMed - indexed for MEDLINE]

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  [Large adrenal cavernous hemangioma as an unexpected finding after work-up of an abdominal mass].

  Fetched: May 26th, 2012, 11:00pm MDT

  [Large adrenal cavernous hemangioma as an unexpected finding after work-up of an abdominal mass].

  Endocrinol Nutr. 2012 Jan;59(1):81-3

  Authors: González Duarte DJ, Serrano Romero M, García-Doncel LG, Baena Nieto G, De Tejada Valdivia PS

  PMID: 21816693 [PubMed - indexed for MEDLINE]

• 

  [ACTH-independent macronodular adrenal hyperplasia and subclinical Cushing's syndrome].

  Fetched: May 26th, 2012, 11:00pm MDT

  [ACTH-independent macronodular adrenal hyperplasia and subclinical Cushing's syndrome].

  Med Clin (Barc). 2012 Feb 4;138(2):89-90

  Authors: Cortés-Berdonces M, Avilés-Pérez MD, García-Martín A, Muñoz-Torres M

  PMID: 21703642 [PubMed - indexed for MEDLINE]

• 

  Resolution of insulin-requiring diabetes in a liver transplant recipient after treatment of a pheochromocytoma: case report and review of literature.

  Fetched: May 24th, 2012, 11:00pm MDT

  Resolution of insulin-requiring diabetes in a liver transplant recipient after treatment of a pheochromocytoma: case report and review of literature.

  Minerva Endocrinol. 2011 Dec;36(4):341-5

  Authors: Gallagher EJ, Courgi R, Heiba S, Tamler R

  Abstract
  The aim of this paper was to report the case of type 2 diabetes and significant insulin resistance that improved dramatically after removal of a pheochromocytoma in a liver transplant recipient , and to provide a review of the relevant literature. We describe the clinical presentation, diagnostic results and management of the patient. In addition, we performed a PubMed search for related English language articles, to provide an overview of the pertinent literature. A 53 year old woman with a history of an orthotopic liver transplantation and insulin-requiring type 2 diabetes was admitted to the hospital with fever, diaphoresis, tachycardia and hypertension. A pheochromocytoma was diagnosed and removed. The patient subsequently developed hypoglycemia and required no further insulin therapy. Pheochromocytomas have been described to lead to hyperglycemia and diabetes, due to the suppression of insulin release and increased insulin resistance. Furthermore, a review of the literature revealed only 3 other reported cases of pheochromocytomas in organ transplant recipients. None of these pheochromocytomas were believed to have occurred de novo after transplantation. This is the first report of a pheochromocytoma in a liver transplant recipient and possibly the first case of a de novo pheochromocytoma in any organ transplant recipient. Moreover, this case showcases pheochromocytomas as a rare cause of diabetes mellitus.
  

  PMID: 22322656 [PubMed - indexed for MEDLINE]

• 

  Surgical management of adrenal metastases.

  Fetched: May 24th, 2012, 11:00pm MDT

  Surgical management of adrenal metastases.

  Langenbecks Arch Surg. 2012 Feb;397(2):179-94

  Authors: Sancho JJ, Triponez F, Montet X, Sitges-Serra A

  Abstract
  PURPOSE: This paper aims to review controversies in the management of adrenal gland metastasis and to reach an evidence-based consensus.
  MATERIALS AND METHODS: A review of English-language studies addressing the management of adrenal metastasis, including indications for surgery, diagnostic imaging, fine-needle aspiration, surgical approach, and outcome was carried out. Results were discussed at the 2011 Workshop of the European Society of Endocrine Surgeons devoted to adrenal malignancies and a consensus statement agreed.
  RESULTS: Patients should be managed by a multidisciplinary team. Positron emission tomography coupled with computed tomography (PET/CT) scanning is the technique of choice for suspected adrenal metastasis. When PET/CT is not available or results are inconclusive, the CT scan or magnetic resonance imaging can be used. Patients should undergo complete hormonal evaluation. Adrenal biopsy should be reserved for cases in which the results of non-invasive techniques are equivocal. If malignancy has been reliably ruled out, patients with adrenal incidentalomas should be managed like noncancer patients.
  CONCLUSIONS: A patient with suspected adrenal metastasis should be considered a candidate for adrenalectomy when: (a) control of extra-adrenal disease can be accomplished, (b) metastasis is isolated to the adrenal gland(s), (c) adrenal imaging is highly suggestive of metastasis or the patient has a biopsy-proven adrenal malignancy, (d) metastasis is confined to the adrenal gland as assessed by a recent imaging study, and (e) the patient's performance status warrants an aggressive approach. In properly selected patients, laparoscopic (or retroperitoneoscopic) adrenalectomy is a feasible and safe option.
  

  PMID: 22205385 [PubMed - indexed for MEDLINE]

• 

  Positional statement of the European Society of Endocrine Surgeons (ESES) on malignant adrenal tumors.

  Fetched: May 24th, 2012, 11:00pm MDT

  Positional statement of the European Society of Endocrine Surgeons (ESES) on malignant adrenal tumors.

  Langenbecks Arch Surg. 2012 Feb;397(2):145-6

  Authors: Henry JF, Peix JL, Kraimps JL

  PMID: 22203016 [PubMed - indexed for MEDLINE]

• 

  Clinical impact of TP53 alterations in adrenocortical carcinomas.

  Fetched: May 24th, 2012, 11:00pm MDT

  Clinical impact of TP53 alterations in adrenocortical carcinomas.

  Langenbecks Arch Surg. 2012 Feb;397(2):209-16

  Authors: Waldmann J, Patsalis N, Fendrich V, Langer P, Saeger W, Chaloupka B, Ramaswamy A, Fassnacht M, Bartsch DK, Slater EP

  Abstract
  BACKGROUND: To evaluate the role of somatic TP53 mutations and to correlate somatic and germline mutations with results of immunostaining, a large cohort of ACC patients was analyzed.
  PATIENTS AND METHODS: Patients with ACC who underwent potential curative surgery at the authors' department were screened for TP53 somatic and germline mutations in exons 5, 6, 7, 8, and 10 by DHPLC analysis. Aberrant samples were further analyzed by direct sequencing. Immunostaining was performed on corresponding paraffin sections in all patients. Complete clinical and follow-up data were correlated with the status of TP53.
  RESULTS: Thirty ACC patients were included. Four of 30 patients showed aberrant DHPLC configuration and direct sequencing confirmed 2 (7%) germline mutations (R337H, R248W), 1 (3%) somatic mutation (R213X), and 1 (3%) noncoding polymorphism (g.17708 A>T). The only patient with a positive family history harbored a TP53 mutation. Tumors of the three patients with mutations showed aberrant p53 expression in more than 10% of cells by immunostaining, compared to only 3 of 27 patients without mutations (p = 0.009). Aberrant p53 expression (>5%) was detected in 12/30 (40%) ACCs. The latter was associated with an increased Ki67 and van Slooten index (p ≤ 0.001; p = 0.020). Disease-free survival decreased significantly in patients with aberrant p53 IHC of more than 5% of cells (65.7 ± 12.4 vs. 26.6 ± 8.7 months; p = 0.043 log rank test).
  CONCLUSIONS: Patients with ACC revealed aberrant expression of p53 in 40%, and mutations were identified in 25% of these patients. Therefore aberrant p53 expression should be considered an indicator for genetic testing. A subgroup of apparently sporadic ACC is caused by TP53 germline mutations, and family history is a strong indicator for p53 germline mutations.
  

  PMID: 22203015 [PubMed - indexed for MEDLINE]

• 

  Presenting multiple adhesion ligands on hydrogels for control of PC12 phenotype.

  Fetched: May 24th, 2012, 11:00pm MDT

  Presenting multiple adhesion ligands on hydrogels for control of PC12 phenotype.

  J Control Release. 2011 Nov 30;152 Suppl 1:e219-20

  Authors: Lee JW, Lee KY

  PMID: 22195867 [PubMed - indexed for MEDLINE]

• 

  Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava--a European Society of Endocrine Surgeons (ESES) survey.

  Fetched: May 24th, 2012, 11:00pm MDT

  Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava--a European Society of Endocrine Surgeons (ESES) survey.

  Langenbecks Arch Surg. 2012 Feb;397(2):225-31

  Authors: Mihai R, Iacobone M, Makay O, Moreno P, Frilling A, Kraimps JL, Soriano A, Villar del Moral J, Barczynski M, Durán MC, Sadler GP, Niederle B, Dralle H, Harrison B, Carnaille B

  Abstract
  BACKGROUND: Most patients with adrenocortical cancer (ACC) continue to present with advanced disease. Invasion into the inferior vena cava (IVC) defines stage III disease and the management of such patients raises additional difficulties.
  METHOD: A multicentre survey was organized by emailing a standardized proforma to members of the European Society of Endocrine Surgery (ESES). Anonymised retrospective clinical data were collected.
  RESULTS: Replies were received from 18 centres in nine countries. ACC with IVC invasion was encountered in 38 patients (18F:20M, age 15-84 years, median 54 years). There were 16 nonfunctioning tumours and 22 functioning tumours predominantly right-sided (26R:12L) and measuring 18-255 mm (median 115 mm). Fourteen patients had metastatic disease at presentation. Tumour thrombus extended in the prehepatic IVC (n = 21), subdiaphragmatic IVC (n = 6) or into the SVC/right atrium (n = 3). Open adrenalectomy was associated with resection of surrounding viscera in 24 patients (nephrectomy n = 16, liver resection n = 14, splenectomy n = 3, Whipple procedure n = 2). IVC was controlled locally (n = 27), at suprahepatic levels (n = 6) or necessitated cardiac bypass (n = 5). Complete resection (R0, n = 20) was achieved in the majority of patients, with a minority having microscopic persistent disease (R1, n = 7) or macroscopic residual disease (R2, n = 4). Perioperative 30-day mortality was 13% (n = 5). Postoperative Mitotane was used in 23 patients and chemotherapy in eight patients. Twenty-five patients died 2-61 months after their operation (median 5 months). Currently, 13 patients are alive at 2-58 months (median 16 months) with known metastatic disease (n = 7) or with no signs of distant disease (n = 6).
  CONCLUSION: This dataset is limited by the lack of a denominator as it remains unknown how many other patients with ACC presenting with IVC invasion did not undergo surgery. The relatively low perioperative mortality and the long disease-free survival achieved by some patients should encourage surgeons with adequate experience to offer surgical treatment to patients presenting with advanced adrenocortical cancers.
  

  PMID: 22134748 [PubMed - indexed for MEDLINE]

• 

  Malignant pheochromocytomas and paragangliomas: a diagnostic challenge.

  Fetched: May 24th, 2012, 11:00pm MDT

  Malignant pheochromocytomas and paragangliomas: a diagnostic challenge.

  Langenbecks Arch Surg. 2012 Feb;397(2):155-77

  Authors: Gimm O, DeMicco C, Perren A, Giammarile F, Walz MK, Brunaud L

  Abstract
  INTRODUCTION: Malignant pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare disorders arising from the adrenal gland, from the glomera along parasympathetic nerves or from paraganglia along the sympathetic trunk. According to the WHO classification, malignancy of PCCs and PGLs is defined by the presence of metastases at non-chromaffin sites distant from that of the primary tumor and not by local invasion. The overall prognosis of metastasized PCCs/PGLs is poor. Surgery offers currently the only change of cure. Preferably, the discrimination between malignant and benign PCCs/PGLs should be made preoperatively.
  METHODS: This review summarizes our current knowledge on how benign and malignant tumors can be distinguished.
  CONCLUSION: Due to the rarity of malignant PCCs/PGLs and the obvious difficulties in distinguishing benign and malignant PCCs/PGLs, any patient with a PCC/PGL should be treated in a specialized center where a multidisciplinary setting with specialized teams consisting of radiologists, endocrinologist, oncologists, pathologists and surgeons is available. This would also facilitate future studies to address the existing diagnostic and/or therapeutic obstacles.
  

  PMID: 22124609 [PubMed - indexed for MEDLINE]

• 

  Paraganglioma: not just an extra-adrenal pheochromocytoma.

  Fetched: May 24th, 2012, 11:00pm MDT

  Paraganglioma: not just an extra-adrenal pheochromocytoma.

  Langenbecks Arch Surg. 2012 Feb;397(2):247-53

  Authors: Laird AM, Gauger PG, Doherty GM, Miller BS

  Abstract
  PURPOSE: Pheochromocytoma (PCC) and paraganglioma (PG) are evaluated and treated similarly. This study evaluates the hypothesis that tumor characteristics and outcome of patients with PCC and PG are equivalent.
  METHODS: Records of patients from a single institution undergoing resection of PCC or PG from 1999 to 2010 were reviewed. Data were collected for demographics, operative records, laboratory and pathologic results, adjuvant and palliative therapy given, recurrence, and length of survival. Descriptive statistics were used to describe differences between patients with benign and malignant PCC and PG. Analysis was performed using the Wilcoxon-Mann-Whitney test with p = 0.05 considered as significant.
  RESULTS: One hundred fifteen patients were identified (106 PCC and nine PG). Of the tumors, 5.2% were bilateral and 10.4% were malignant. Forty-three of the 115 patients underwent genetic testing; 21 out of 37 (56.8%) PCC and five out of six (83.3%) PG had a genetic mutation. Twelve patients (seven PCC and five PG) had malignant tumors. Malignant PG (mPG) exhibited more invasive pathologic characteristics. The median sizes of benign and malignant PCC (mPCC) were 4.0 (0.7-14 cm) and 5.5 cm (3.7-11.2 cm), respectively, p = 0.03. The median sizes of benign and mPG were 4.1 (2.7-5.4 cm) and 5.8 cm (4-6.2 cm), respectively, p = 0.11. Sites of recurrence were similar between the groups. Patients with mPG received chemotherapy more often than those with mPCC. With a median follow-up of 54.7 months (2.0-185.3), two out of five mPG and zero out of seven mPCC had died of the disease.
  CONCLUSION: Tumor size does not appear to correlate with malignancy in a clinically significant manner. Malignant paraganglioma may be more aggressive than malignant pheochromocytoma and is frequently offered more adjuvant therapy. PCC and PG should be evaluated separately in future analyses of these diseases.
  

  PMID: 22086065 [PubMed - indexed for MEDLINE]

• 

  Adrenocortical carcinoma: effect of hospital volume on patient outcome.

  Fetched: May 24th, 2012, 11:00pm MDT

  Adrenocortical carcinoma: effect of hospital volume on patient outcome.

  Langenbecks Arch Surg. 2012 Feb;397(2):201-7

  Authors: Lombardi CP, Raffaelli M, Boniardi M, De Toma G, Marzano LA, Miccoli P, Minni F, Morino M, Pelizzo MR, Pietrabissa A, Renda A, Valeri A, De Crea C, Bellantone R

  Abstract
  PURPOSE: Optimal management of adrenocortical carcinoma (ACC) involves a detailed diagnostic workup, radical surgery, and appropriate adjuvant therapy. However, due to the rarity of this disease, adequate expertise is necessary to ensure optimal patient care. We evaluated if the experience of a treating center influences the outcome of ACC.
  METHODS: Two hundred sixty-three patients who underwent adrenalectomy for ACC were included in a multi-institutional surgical survey and divided into 2 groups: "high-volume center" (HVC) (≥10 adrenalectomies for ACC) and "low-volume center" (LVC) (<10 adrenalectomies for ACC). A comparative analysis was performed.
  RESULTS: One hundred seventy-two patients underwent adrenalectomy at HVC and 91 at LVC. The two groups were homogeneous for age, sex, clinical presentation, and stage. The mean lesions size of ACC was higher in HVC than in LVC (104.1 ± 54.6 vs 82.8 ± 41.3 mm; P < 0.001). A significantly higher rate of lymph node dissection (P < 0.01) and of multiorgan resection (P < 0.01) was accomplished in HVC. The number of patients who underwent adjuvant therapy was significantly higher in HVC (P < 0.001). Local recurrence rate was lower in patients treated at HVC (6% vs 18.5%; P = NS). Mean time to recurrence was significantly longer in HVC than in LVC (25.2 ± 28.1 vs 10.1 ± 7.5; P < 0.01).
  CONCLUSION: The expertise of dedicated centers had a positive impact on the outcome of patients with ACC, resulting in a lower recurrence rate and improved mean time to recurrence. The improved patient outcome could be related not only to the appropriateness of the surgical procedure, but also to a more adequate multidisciplinary approach.
  

  PMID: 22069043 [PubMed - indexed for MEDLINE]

• 

  Malignant pheochromocytoma: new malignancy criteria.

  Fetched: May 24th, 2012, 11:00pm MDT

  Malignant pheochromocytoma: new malignancy criteria.

  Langenbecks Arch Surg. 2012 Feb;397(2):239-46

  Authors: de Wailly P, Oragano L, Radé F, Beaulieu A, Arnault V, Levillain P, Kraimps JL

  Abstract
  PURPOSE: The pathological diagnosis of malignancy in pheochromocytomas remains a controversial issue. According to the WHO, malignancy is defined in the presence of metastasis. Multiparameter scoring systems such as PASS (Pheochromocytoma of Adrenal gland Scaled Score) have been used but remain controversial. The aim of this study was to search for new immunohistologic elements allowing determination of pheochromocytoma malignancy.
  METHODS: Among 53 patients operated for pheochromocytoma between 1993 and 2009, we selected pheochromocytomas with proven metastasis, seven cases in group 1 (G1) and paired two others groups: group 2 (G2), patients who had "benign" pheochromocytoma with PASS ≥4 and group 3 (G3), patients who had "benign" pheochromocytoma with PASS <4. We retrospectively analysed PASS criteria, size, weight, tumour necrosis, Ki-67 and pS100 staining.
  RESULTS: The size and weight of the lesion were directly and significantly correlated to malignancy in all three groups: respectively 9.7 cm and 292.0 g (G1), 6.2 cm and 83.8 g (G2) and 3.8 cm and 37.1 g (G3) (p < 0.005 for both). Tumour necrosis (TN) was present in all G1 (p < 0.005) and respectively at 0% and 37.5% in G2 and G3. Ki-67 is directly correlated to presence of TN (p < 0.005) and malignancy (G1 14.1%, G2 1.8%, G3 2.6%; p < 0.001). All G1 had a Ki-67 index >4%, although one G3 presented an 11% Ki-67 index. There was an inverse statistically significant correlation between the three groups in staining using pS100 (p < 0.01).
  CONCLUSIONS: Size and weight of the pheochromocytoma are directly related to PASS and malignancy. The presence of tumour necrosis, Ki-67 index >4% and pS100 absence impose a close histopathological evaluation and follow-up with regard to cases presenting a high risk of malignancy/recurrence.
  

  PMID: 22069042 [PubMed - indexed for MEDLINE]

• 

  Adrenocortical carcinoma: which surgical approach?

  Fetched: May 24th, 2012, 11:00pm MDT

  Adrenocortical carcinoma: which surgical approach?

  Langenbecks Arch Surg. 2012 Feb;397(2):195-9

  Authors: Carnaille B

  Abstract
  INTRODUCTION: There are no randomised studies comparing open and laparoscopic approaches foradrenalectomy in patients with adrenal cortical carcinoma.
  METHODS: There is evidence of postoperative benefit for the patients undergoing laparoscopic adrenalectomy compared to open adrenalectomy (level B).
  RESULTS: Results from comparison of oncological outcomes in ACC between open and laparoscopic approaches are equivocal: increasedrisk of local recurrence and peritoneal carcinomatosis by the laparoscopic route (level D), and identical results between the two approaches in terms of survival, recurrence and peritoneal carcinomatosis (level C).
  CONCLUSION: An open approach is recommended in case of local invasion, with a view to achieving an R0 resection (level D). Laparoscopic resection of ACC/potentially malignant tumours, which includes removal of surrounding periadrenal fat and results in an R0 resection without tumour capsule rupture, may be performed for preoperative and intraoperative stage 1-2 ACC and tumours with a diameter < 10 cm (level C).
  

  PMID: 21947510 [PubMed - indexed for MEDLINE]

• 

  Determinants for malignancy in surgically treated adrenal lesions.

  Fetched: May 24th, 2012, 11:00pm MDT

  Determinants for malignancy in surgically treated adrenal lesions.

  Langenbecks Arch Surg. 2012 Feb;397(2):217-23

  Authors: Wright L, Nordenström E, Almquist M

  Abstract
  PURPOSE: It is difficult to definitively rule out or establish malignancy pre-operatively in patients with adrenal lesions referred for adrenal surgery. The aim of this study was to identify risk factors for a malignant diagnosis in patients treated with adrenalectomy.
  METHODS: Consecutive adrenalectomies at the Department of Surgery, Lund University Hospital between 2000 and 2009 were identified. Pre-operative clinical, radiological and biochemical data, as well as details on the operation and histology, were retrieved from patients' medical records.
  RESULTS: There were 175 adrenal operations in 172 patients (98 female and 77 male). There were 28 malignancies. Malignant tumours were larger than in benign cases (p = 0.003), had radiological signs of malignancy more often (p = 0.001) and the patients were more likely to have a history of malignancy (p = 0.001). There were no differences regarding age, gender, body mass index or mode of detection (incidental vs. clinical) between patients with benign and malignant diagnoses. A unilateral uptake on fluorine-18 deoxyglucose positron emission tomography was more frequent in malignant cases than in benign; however, this association did not reach statistical significance.
  CONCLUSIONS: Size of adrenal lesion, suspicious radiological findings and history of malignancy were factors associated with malignancy.
  

  PMID: 21935703 [PubMed - indexed for MEDLINE]

• 

  Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas.

  Fetched: May 24th, 2012, 11:00pm MDT

  Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas.

  Langenbecks Arch Surg. 2012 Feb;397(2):233-8

  Authors: Alesina PF, Hinrichs J, Meier B, Schmid KW, Neumann HP, Walz MK

  Abstract
  INTRODUCTION: Cortical-sparing adrenalectomy in bilateral pheochromocytomas offers a postoperative corticoid-free course and has to be balanced against the risk of local recurrence. In this study we report our experience with the minimally invasive cortical-sparing adrenalectomy in patients with bilateral pheochromocytomas.
  METHODS: From January 1996 to February 2011, 66 patients (45 men, 21 women; mean age 36 ± 16 years) were treated for bilateral pheochromocytomas. Fifty-seven patients (88%) were affected by genetic diseases. In 32 patients surgery was synchronously performed on both side, in 34 cases adrenalectomy followed previous surgery. All in all, 101 operations (47 right, 54 left) were conducted using the retroperitoneoscopic access (n = 97) or the laparoscopic route (n = 4).
  RESULTS: The mortality in our series was zero. Postoperative complications included one patient with a bleeding requiring reoperation and one patient developing a cerebral stroke on the fifth postoperative day. The mean operative time was 67 ± 26 min for unilateral adrenalectomy and 128 ± 68 min for bilateral surgery (range 25-300 min). A cortical-sparing resection was possible in 89 procedures resulting in a corticoid-free postoperative course in 60 patients (91%). A postoperative corticosteroid substitution therapy was necessary in six patients. During a median follow-up period of 48 months, one patient showed a persistent disease and needed reoperation, none developed a recurrent disease.
  CONCLUSION: Cortical-sparing surgery for bilateral pheochromocytomas has a low recurrence rate and avoids lifelong cortisone substitution therapy in the majority of cases.
  

  PMID: 21935702 [PubMed - indexed for MEDLINE]

• 

  KCNQ1OT1 hypomethylation: A Novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?

  Fetched: May 24th, 2012, 11:00pm MDT

  KCNQ1OT1 hypomethylation: A Novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?

  Pediatr Blood Cancer. 2011 Dec 11;

  Authors: Wijnen M, Alders M, Zwaan CM, Wagner A, Cheng FW, van den Heuvel-Eibrink MM

  Abstract
  Pediatric adrenal tumors, other than neuroblastoma, are rare and can be associated with a genetic predisposition. In this report we describe two patients with an isolated and apparently sporadic adrenocortical tumor; one girl with a carcinoma, the other girl with an adenoma. In both patients genetic screening revealed hypomethylation of the KCNQ1OT1 gene, well-known for its association with the Beckwith-Wiedemann syndrome. This represents a likely novel genetic predisposition in patients with adrenocortical tumors without clear phenotypic features of the Beckwith-Wiedemann syndrome. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc.
  

  PMID: 22610651 [PubMed - as supplied by publisher]

• 

  Characterization of adrenal nodules with dual-energy CT: can virtual unenhanced attenuation values replace true unenhanced attenuation values?

  Fetched: May 24th, 2012, 11:00pm MDT

  Characterization of adrenal nodules with dual-energy CT: can virtual unenhanced attenuation values replace true unenhanced attenuation values?

  AJR Am J Roentgenol. 2012 Apr;198(4):840-5

  Authors: Ho LM, Marin D, Neville AM, Barnhart HX, Gupta RT, Paulson EK, Boll DT

  Abstract
  OBJECTIVE: The purpose of our study was to investigate whether virtual unenhanced adrenal nodule attenuation values can replace true noncontrast attenuation values.
  MATERIALS AND METHODS: Twenty-three incidentally discovered adrenal nodules (19 adenomas and four metastases) were identified in 19 patients (11 men and eight women; mean age, 65 years; age range, 38-84 years) who underwent unenhanced single-energy CT followed by contrast-enhanced dual-energy CT on the same scanner. A virtual unenhanced imaging dataset was generated from each dual-energy CT dataset. CT attenuation of each adrenal nodule was measured at the same location on virtual unenhanced images and true unenhanced images by three radiologists and mean values compared using the Student t test. Correlation between virtual unenhanced and true unenhanced values was determined using linear regression analysis. The mean difference and percentage of diagnostic agreement were also determined. Interreader variability was assessed using the intraclass correlation coefficient (ICC).
  RESULTS: The mean ± SD attenuation values for virtual unenhanced images and true unenhanced images were 14.7 ± 15.1 HU and 12.9 ± 13.4 HU, respectively (p = 0.2). Strong positive correlation was observed between virtual unenhanced images and true unenhanced images (R = 0.83-0.87). The mean difference between virtual unenhanced images and true unenhanced images was 1.8 ± 1.7 HU. Diagnostic agreement between virtual unenhanced images and true unenhanced images was 83-91% for three radiologists. No malignant nodules were misclassified as benign on virtual unenhanced images. The ICC was 0.88 and 0.96 for virtual unenhanced images and true unenhanced images, respectively, indicating high interreader agreement.
  CONCLUSION: Virtual unenhanced and true unenhanced attenuation measurements of adrenal nodules were not significantly different and showed strongly positive linear correlation. This finding resulted in substantial diagnostic agreement between virtual unenhanced images and true unenhanced images for distinguishing benign from malignant nodules.
  

  PMID: 22451549 [PubMed - indexed for MEDLINE]

• 

  Catecholamine-induced cardiomyopathy and paraganglioneuroma in a pediatric patient.

  Fetched: May 20th, 2012, 11:00pm MDT

  Catecholamine-induced cardiomyopathy and paraganglioneuroma in a pediatric patient.

  Anadolu Kardiyol Derg. 2011 Dec;11(8):743-4

  Authors: Narin N, Baykan A, Sezer S, Onan SH, Uzüm K, Küçükaydın M

  PMID: 22137946 [PubMed - indexed for MEDLINE]

• 

  Editorial comment.

  Fetched: May 20th, 2012, 11:00pm MDT

  Editorial comment.

  Urology. 2012 Mar;79(3):583; author reply 583-4

  Authors: Autorino R, De Sio M

  PMID: 22386403 [PubMed - indexed for MEDLINE]

• 

  Comparison of single-surgeon series of transperitoneal laparoendoscopic single-site surgery and standard laparoscopic adrenalectomy.

  Fetched: May 20th, 2012, 11:00pm MDT

  Comparison of single-surgeon series of transperitoneal laparoendoscopic single-site surgery and standard laparoscopic adrenalectomy.

  Urology. 2012 Mar;79(3):577-83

  Authors: Wang L, Liu B, Wu Z, Yang Q, Chen W, Sheng H, Xu Z, Xiao L, Wang C, Sun Y

  Abstract
  OBJECTIVE: To assess the feasibility, safety, and efficacy of transperitoneal laparoendoscopic single-site (LESS) adrenalectomy and determine whether it shows any objective advantage compared with standard laparoscopy.
  METHODS: From August 2009 to May 2011, 13 transperitoneal LESS adrenalectomies were performed through a 2-3-cm skin incision using the TriPort access system. This cohort was compared with a contemporary 1:2 matched-pair group of 26 patients undergoing standard laparoscopic adrenalectomy by the same urologist. The perioperative outcomes, including cosmetic satisfaction scores, were statistically analyzed.
  RESULTS: The 2 groups were comparable with respect to patient demographics, estimated blood loss, and postoperative hospitalization (P > .05). The LESS procedures had a longer mean operative time (148.5 vs 112.9 minutes, P = .032) but a significantly lower postoperative visual analog pain scale score (2.3 vs 3.7, P = .001), fewer patients requiring analgesics (30.8% vs 73.1%, P = .011), and an earlier resumption of oral intake (21.6 vs 26.0 hours, P = .002). The mean length of the scar in the LESS group was much smaller (2.3 vs 5.9 cm, P < .0001) with a statistically significant greater mean cosmetic satisfaction score (9.5 vs 9.1, P = .042).
  CONCLUSION: The perioperative outcomes of transperitoneal LESS adrenalectomy for small adrenal tumors were comparable to those with the standard laparoscopic approach. It also provides better postoperative pain control, faster recovery of bowel function, and better cosmetic satisfaction than standard laparoscopy, albeit with a longer operative time.
  

  PMID: 22386401 [PubMed - indexed for MEDLINE]

• 

  Mucinous adenocarcinoma developed from human fallopian tube epithelial cells through defined genetic modifications.

  Fetched: May 19th, 2012, 11:00pm MDT

  Mucinous adenocarcinoma developed from human fallopian tube epithelial cells through defined genetic modifications.

  Cell Cycle. 2012 Jun 1;11(11)

  Authors: Shan W, Mercado-Uribe I, Zhang J, Rosen D, Zhang S, Wei J, Liu J

  Abstract
  Recent studies have suggested that some ovarian and pelvic serous carcinomas could originate from the fimbriated end of the distal fallopian tube. To test this hypothesis, we immortalized a normal human fallopian tube epithelial (FTE) cell line by using retrovirus-mediated overexpression of the early region of the SV40 T/t antigens and the human telomerase reverse transcriptase subunit (hTERT). These immortalized FTEs were then transformed by ectopic expression of oncogenic human HRAS (V12) . Tumorigenicity of the immortalized and/or transformed cells was subsequently tested by anchorage-independence growth assay and inoculation into nude mice via subcutaneous and intraperitoneal injection. As expected, the HRAS (V12) -transformed FTEs produced tumors through both subcutaneous and intraperitoneal injections, whereas no tumor growth was observed in immortalized FTEs. Unexpectedly, histopathological examination of tumors resulting from subcutaneous as well as intraperitoneal injections revealed largely poorly differentiated mucinous adenocarcinoma mixed with undifferentiated carcinoma. The tumor implants invaded extensively to the liver, colon, spleen, omentum, adrenal gland and renal capsule. Immunohistochemical staining of tumor cells showed positive staining for the epithelial cell markers cytokeratin AE1/AE3 and Müllerian lineage marker PAX8. Our study demonstrates that FTEs can generate poorly differentiated mucinous adenocarcinoma mixed with undifferentiated carcinoma through genetic modifications. Thus, we provide the first experimental evidence that fimbrial epithelial cells of the fallopian tube could be a potential source of ovarian mucinous adenocarcinoma.
  

  PMID: 22592533 [PubMed - as supplied by publisher]

• 

  An unusual cause of hyperandrogenism.

  Fetched: May 19th, 2012, 11:00pm MDT

  An unusual cause of hyperandrogenism.

  Neth J Med. 2012 Jan;70(1):41, 45

  Authors: Wendker-van Wattum M, Wouters RS, van der Wal JE, Glaudemans AW, Wolffenbuttel BH

  PMID: 22271814 [PubMed - indexed for MEDLINE]

• 

  Castleman's disease mimicking right adrenal neoplasm: a case report.

  Fetched: May 19th, 2012, 11:00pm MDT

  Castleman's disease mimicking right adrenal neoplasm: a case report.

  Turk J Gastroenterol. 2011 Oct;22(5):551-4

  Authors: Çolakoğlu T, Ezer A, Koçer E, Yildirim S, Bellı S

  Abstract
  Castleman's disease usually manifests as a solitary mediastinal tumor and only rarely as an isolated retroperitoneal mass. This disorder is often undiagnosed or misdiagnosed. Thus, only very few patients have been reported and little information is available in the literature. The definitive diagnosis is based on postoperative pathological findings. We report a case of a 57-year-old female with a Castleman's tumor located superomedial to the upper pole of the right kidney that mimicked an adrenal neoplasm. The mass was surgically resected, and the histopathological diagnosis of the resected tissue was hyaline-vascular type of Castleman's disease. Although retroperitoneal Castleman's disease is rare, it should be considered in the differential diagnosis of retroperitoneal masses.
  

  PMID: 22234768 [PubMed - indexed for MEDLINE]

• 

  Bilateral primary adrenal lymphoma accompanying hypertension.

  Fetched: May 19th, 2012, 11:00pm MDT

  Bilateral primary adrenal lymphoma accompanying hypertension.

  Urology. 2012 Feb;79(2):e27-8

  Authors: Wang Q, Cao X, Jiang J, Wang T, Jin MS

  Abstract
  Primary adrenal lymphoma (PAL) accompanied by hypertension is extremely rare. We present a case of PAL with hypertension, whom was treated with bilateral adrenalectomy and a combination of the modified Appleby operation and chemotherapy. Computed tomography and biopsy is helpful to aid diagnosis.
  

  PMID: 21862117 [PubMed - indexed for MEDLINE]

• 

  Adrenocortical carcinoma masquerading as a benign adenoma on computed tomography washout study.

  Fetched: May 19th, 2012, 11:00pm MDT

  Adrenocortical carcinoma masquerading as a benign adenoma on computed tomography washout study.

  Urology. 2012 Feb;79(2):e19-20

  Authors: Simhan J, Canter D, Teper E, Smaldone MC, Patil N, Patchefsky A, Guttmann MC, Milestone B, Wong YN, Hicks LA, Uzzo RG, Kutikov A

  Abstract
  An incidental adrenal mass is a common finding on cross-sectional imaging, with most of these lesions being benign adenomas. Indications for adrenalectomy turn on the likelihood that a mass is malignant or whether it exhibits metabolic activity. Modern imaging is considered highly accurate in differentiating adrenal adenomas from other adrenal pathology. We present a case of a 5-cm adrenal lesion with computed tomography washout characteristics consistent with a benign adenoma, which proved upon resection to be an adrenocortical carcinoma.
  

  PMID: 21813169 [PubMed - indexed for MEDLINE]

• 

  The glucocorticoid receptor and its expression in the anterior pituitary and the adrenal cortex: a source of variation in hypothalamic-pituitary-adrenal axis function; implications for pituitary and adrenal tumors.

  Fetched: May 19th, 2012, 11:00pm MDT

  The glucocorticoid receptor and its expression in the anterior pituitary and the adrenal cortex: a source of variation in hypothalamic-pituitary-adrenal axis function; implications for pituitary and adrenal tumors.

  Endocr Pract. 2011 Nov-Dec;17(6):941-8

  Authors: Briassoulis G, Damjanovic S, Xekouki P, Lefebvre H, Stratakis CA

  Abstract
  OBJECTIVE: To review the expression of the glucocorticoid receptor (GR) in anterior pituitary and adrenocortical cells and tumors derived from these tissues as well as factors that may influence its expression.
  METHODS: We present an overview of the relevant literature, with a focus on data generated from our studies.
  RESULTS: The expression of the GR is an essential element of the negative feedback that closes the loop formed by corticotropin-releasing hormone, adrenocorticotropic hormone, and cortisol in the context of the hypothalamic-pituitary-adrenal (HPA) axis. Although the GR expression in anterior pituitary cells-and in particular the corticotrophs-was first demonstrated several years ago, it was not known until relatively recently where, by what cells, and in what form the GR is expressed in the adrenal cortex. The variability in the expression of the GR in pituitary and adrenocortical cells may underlie the substantial differences in HPA axis function across individuals, especially when testing for tumors associated with hypercortisolemia. This expression is influenced by a multitude of tissue-specific factors, which may explain why it is so difficult to interpret (or reproduce) studies that are based on GR functional polymorphisms on different cohorts of patients or even different sets of laboratory animals.
  CONCLUSION: This review highlights the variability in expression and function of the GR in pituitary and adrenocortical cells as one of the reasons for the appreciable differences in HPA axis function across individuals. Particular attention was paid to interactions that may affect the interpretation of diagnostic testing of the HPA axis in patients with pituitary adenomas (Cushing disease) or adrenocortical tumors (Cushing syndrome).
  

  PMID: 21742609 [PubMed - indexed for MEDLINE]

• 

  Acquisition Order of Ras and p53 Gene Alterations Defines Distinct Adrenocortical Tumor Phenotypes.

  Fetched: May 18th, 2012, 11:00pm MDT

  Acquisition Order of Ras and p53 Gene Alterations Defines Distinct Adrenocortical Tumor Phenotypes.

  PLoS Genet. 2012 May;8(5):e1002700

  Authors: Herbet M, Salomon A, Feige JJ, Thomas M

  Abstract
  Sporadic adrenocortical carcinomas (ACC) are rare endocrine neoplasms with a dismal prognosis. By contrast, benign tumors of the adrenal cortex are common in the general population. Whether benign tumors represent a separate entity or are in fact part of a process of tumor progression ultimately leading to an ACC is still an unresolved issue. To this end, we have developed a mouse model of tumor progression by successively transducing genes altered in adrenocortical tumors into normal adrenocortical cells. The introduction in different orders of the oncogenic allele of Ras (H-Ras(G12V)) and the mutant p53(DD) that disrupts the p53 pathway yielded tumors displaying major differences in histological features, tumorigenicity, and metastatic behavior. Whereas the successive expression of Ras(G12V) and p53(DD) led to highly malignant tumors with metastatic behavior, reminiscent of those formed after the simultaneous introduction of p53(DD) and Ras(G12V), the reverse sequence gave rise only to benign tumors. Microarray profiling revealed that 157 genes related to cancer development and progression were differentially expressed. Of these genes, 40 were up-regulated and 117 were down-regulated in malignant cell populations as compared with benign cell populations. This is the first evidence-based observation that ACC development follows a multistage progression and that the tumor phenotype is directly influenced by the order of acquisition of genetic alterations.
  

  PMID: 22589739 [PubMed - in process]

• 

  Characterization of two mouse models of metastatic pheochromocytoma using bioluminescence imaging.

  Fetched: May 18th, 2012, 11:00pm MDT

  Characterization of two mouse models of metastatic pheochromocytoma using bioluminescence imaging.

  Cancer Lett. 2012 Mar;316(1):46-52

  Authors: Giubellino A, Woldemichael GM, Sourbier C, Lizak MJ, Powers JF, Tischler AS, Pacak K

  Abstract
  Pheochromocytoma is the most common tumor of the adrenal medulla in adults. The lack of sensitive animal models of pheochromocytoma has hindered the study of this tumor and in vivo evaluation of antitumor agents. In this study we generated two sensitive luciferase models using bioluminescent pheochromocytoma cells: an experimental metastasis model to monitor tumor spreading and a subcutaneous model to monitor tumor growth and spontaneous metastasis. These models offer a platform for sensitive, non-invasive and real-time monitoring of pheochromocytoma primary growth and metastatic burden to follow the course of tumor progression and for testing relevant antitumor treatments in metastatic pheochromocytoma.
  

  PMID: 22154086 [PubMed - indexed for MEDLINE]

• 

  Hypertensive retinopathy in a child caused by pheochromocytoma: identification after a failed school vision screening.

  Fetched: May 17th, 2012, 11:00pm MDT

  Hypertensive retinopathy in a child caused by pheochromocytoma: identification after a failed school vision screening.

  J AAPOS. 2012 Feb;16(1):97-9

  Authors: Tibbetts MD, Wise R, Forbes B, Hedrick HL, Levin AV

  Abstract
  A 7-year-old girl was referred for ophthalmological examination after the result of a routine school vision screening identified unilateral vision loss. Fundus examination showed bilateral but markedly asymmetric macular exudates and optic disk edema. After the results of two blood pressure measurements were within normal limits, a third markedly elevated measurement revealed malignant hypertension and led to a diagnosis of pheochromocytoma, a rare catecholamine-secreting tumor. The tumor was resected, and 6 months later the patient's blood pressure had normalized; however, although visual acuity had improved to 20/20 in the right eye, it remained 20/200 in the left eye, with decreased disk edema but the persistence of the macular exudates. The identification of an abdominal malignancy through a school vision screening may have saved this child's life. The need for repeated blood pressure measurement is also highlighted.
  

  PMID: 22245022 [PubMed - indexed for MEDLINE]

• 

  CEUS of adrenal mass lesions--the break-through?

  Fetched: May 17th, 2012, 11:00pm MDT

  CEUS of adrenal mass lesions--the break-through?

  Ultraschall Med. 2011 Oct;32(5):437-9

  Authors: Mostbeck G

  PMID: 21986924 [PubMed - indexed for MEDLINE]

• 

  Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review.

  Fetched: May 17th, 2012, 11:00pm MDT

  Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review.

  Intern Med. 2011;50(13):1419-24

  Authors: Saito T, Tojo K, Furuta N, Ono K, Sasano H, Utsunomiya K

  Abstract
  We report a 61-year-old male with gynecomastia, poor libido and erectile dysfunction. Endocrinological studies showed high levels of estradiol and dehydroepiandrosterone sulfate. Although luteinizing hormone (LH) level was within the normal limit, the concentration of follicle-stimulating hormone (FSH) was under the normal limit. Delayed response of LH and poor response of FSH to gonadotropin-releasing hormone administration were detected. Magnetic resonance imaging of the abdomen revealed a left adrenal tumor. Although the surgically-resected tumor was diagnosed as a high grade ACC based on Weiss's criteria of adrenocortical malignancy, no metastasis was detected. Since estrogen levels normalized after resection, feminizing ACC was confirmed. While LH concentration increased slightly after operation, FSH level became transiently elevated over the normal limit, and finally reached the normal range. These data may suggest that FSH was suppressed selectively by hormone produced by ACC different from estrogen.
  

  PMID: 21720063 [PubMed - indexed for MEDLINE]

• 

  Differentiation between benign and malignant adrenal mass using contrast-enhanced ultrasound.

  Fetched: May 17th, 2012, 11:00pm MDT

  Differentiation between benign and malignant adrenal mass using contrast-enhanced ultrasound.

  Ultraschall Med. 2011 Oct;32(5):460-71

  Authors: Friedrich-Rust M, Glasemann T, Polta A, Eichler K, Holzer K, Kriener S, Herrmann E, Nierhoff J, Bon D, Bechstein WO, Vogl T, Zeuzem S, Bojunga J

  Abstract
  PURPOSE: Adrenal masses can be detected by ultrasound with high sensitivity and specificity. The aim of the present study was to evaluate CEUS in a large patient population using CEUS patterns identified in a previous pilot study.
  MATERIALS AND METHODS: 116 adrenal masses were evaluated by ultrasound, including CEUS with the contrast agent Sonovue®. The dynamic of contrast enhancement (CE) was analyzed using time-intensity curves. The time of the first CE in the adrenal mass was used to define four CEUS patterns: pattern I = early arterial CE, pattern II = arterial CE, pattern III = late CE, pattern IV = no CE. In addition, all patients received CT/MRI and hormonal testing. In suspicious cases biopsy or adrenalectomy was performed.
  RESULTS: CEUS patterns I&II were seen in all patients with primary or secondary malignant lesions of the adrenal gland (n = 16). The sensitivity and specificity of CEUS for the diagnosis of malignant adrenal mass were 100 % (CI [75;100]) and 67 % (CI [56;75]), respectively. Overall histology was available as a reference method for 40 adrenal masses. In 68 % of histologically diagnosed adrenal masses, MRI/CT and CEUS were congruent concerning the characterization of malignant versus benign adrenal mass.
  CONCLUSION: Contrast-enhanced ultrasound may be a useful method in the diagnostic work-up of adrenal mass with excellent sensitivity for the diagnosis of malignancy.
  

  PMID: 21667434 [PubMed - indexed for MEDLINE]

• 

  ULTRASONOGRAPHIC IDENTIFICATION OF VASCULAR INVASION BY ADRENAL TUMORS IN DOGS.

  Fetched: May 17th, 2012, 11:00pm MDT

  ULTRASONOGRAPHIC IDENTIFICATION OF VASCULAR INVASION BY ADRENAL TUMORS IN DOGS.

  Vet Radiol Ultrasound. 2012 May 14;

  Authors: Davis MK, Schochet RA, Wrigley R

  Abstract
  Adrenalectomy is the treatment of choice for adrenal tumors that are producing adverse clinical signs. Surgical planning prior to adrenalectomy is aided by identifying tumors with invasion into adjacent vessels or the presence of a tumor thrombus extending into the caudal vena cava. In this paper, we evaluated the sensitivity and specificity of ultrasound in determining if vascular invasion or tumor thrombus is present. Thirty-four dogs with 36 adrenal tumors were reviewed retrospectively. Overall, 36% of tumors had vascular invasion. Abdominal ultrasound was 100% sensitive and 96% specific in identifying the presence of a tumor thrombus in the caudal vena cava. The sensitivity and specificity was 76% and 96%, respectively, when all forms of vascular invasion were evaluated and included patients with vascular wall invasion without concurrent thrombus. Abdominal ultrasound is a good screening tool for identifying vascular invasion or tumor thrombus associated with adrenal tumors in dogs.
  

  PMID: 22583157 [PubMed - as supplied by publisher]

• 

  Serious gastric ulcer event after stereotactic body radiotherapy (SBRT) delivered with concomitant vinorelbine in a patient with left adrenal metastasis of lung cancer.

  Fetched: May 17th, 2012, 11:00pm MDT

  Serious gastric ulcer event after stereotactic body radiotherapy (SBRT) delivered with concomitant vinorelbine in a patient with left adrenal metastasis of lung cancer.

  Acta Oncol. 2012 May;51(5):624-8

  Authors: Onishi H, Ozaki M, Kuriyama K, Komiyama T, Marino K, Araya M, Saito R, Aoki S, Maehata Y, Tomiaga L, Oguri M, Watanabe I, Onohara K, Sano N, Araki T

  PMID: 22582719 [PubMed - in process]

• 

  Localization of breast cancer resistance protein (Bcrp) in endocrine organs and inhibition of its transport activity by steroid hormones.

  Fetched: May 17th, 2012, 11:00pm MDT

  Localization of breast cancer resistance protein (Bcrp) in endocrine organs and inhibition of its transport activity by steroid hormones.

  Cell Tissue Res. 2012 May 13;

  Authors: Dankers AC, Sweep FC, Pertijs JC, Verweij V, van den Heuvel JJ, Koenderink JB, Russel FG, Masereeuw R

  Abstract
  Breast cancer resistance protein (BCRP) is known for its protective function against the toxic effects of exogenous compounds. In addition to this, a role in the transport of endogenous compounds has been described. Since BCRP in the plasma membrane was shown to be regulated by sex steroids, we investigated the presence and possible role of BCRP in steroid hormone-producing organs. Therefore, the presence and localization of Bcrp was investigated in endocrine organs of wild-type mice. Furthermore, the interaction of various steroid hormones with human BCRP activity was studied. Quantitative PCR revealed Bcrp mRNA in the pituitary and adrenal glands, pancreas, ovary, testis and adipose tissue. Immunohistochemistry revealed the presence of Bcrp in the cortex of the adrenal gland and in plasma membranes of adipocytes. In the pituitary gland, pancreas, ovary and testis, Bcrp was mainly located in the capillaries. The interaction between BCRP and 12 steroid hormones was studied using membrane vesicles of HEK293-BCRP cells. Estradiol, testosterone, progesterone and androstenedione inhibited BCRP-mediated uptake of (3)H-estrone sulphate (E(1)S) most potently, with calculated inhibitory constant (Ki) values of 5.0 ± 0.2, 36 ± 14, 14.7 ± 1.3 and 217 ± 13 μM, respectively. BCRP function was attenuated non-competitively, which implies an allosteric inhibition of BCRP-mediated E(1)S transport by these steroids. In conclusion, localization of Bcrp in endocrine organs together with the efficient allosteric inhibition of the efflux pump by steroid hormones are suggestive for a role for BCRP in steroid hormone regulation.
  

  PMID: 22581381 [PubMed - as supplied by publisher]

• 

  Transcriptional silencing of the inhibin-α gene in human gastric carcinoma cells.

  Fetched: May 17th, 2012, 11:00pm MDT

  Transcriptional silencing of the inhibin-α gene in human gastric carcinoma cells.

  Int J Oncol. 2012 May 10;

  Authors: Kim YI, Shim J, Kim BH, Lee SJ, Lee HK, Cho C, Cho BN

  Abstract
  Although inhibin was first identified as a hormone regulating pituitary FSH secretion, it was later recognized to act as a tumor suppressor in the gonad and adrenal glands. Recently, the alpha subunit of this dimeric hormone (inhibin‑α) was reported to be involved in prostate tumorigenesis. To identify additional roles outside the reproductive axis, we investigated inhibin‑α gene activity and subsequent cell fate in human gastric cancer cells. The results were as follows: all the gastric cancer cells had at least one of a set of abnormalities including hypermethylation of the promoter, mutation of the 5'‑UTR or allelic imbalance including LOH in the inhibin‑α gene. Hypermethylation of the promoter and mutation of the 5'‑UTR in inhibin‑α were observed in SNU‑1, SNU‑5 and SNU‑484 cells. LOH was observed in AGS, KATO III, SNU‑5, SNU‑484 and SNU‑668 cells. Treatment with 5‑AzaC, a demethylating agent, induced demethylation of the inhibin‑α promoter in the SNU‑1, SNU‑5 and SNU‑484 cell lines, with the CpG5 site being strongly influenced by 5‑AzaC. In addition, inhibin‑α mRNA and protein were maintained at low levels in most of the gastric cancer cell lines. These low levels of mRNA and protein expression could be increased in most lines by treatment with 5‑AzaC. These increased inhibin‑α expression levels seemed to be closely associated with apoptosis and suppression of cell growth. Taken together, our results reveal that the inhibin‑α gene is transcriptionally silenced in human gastric cancer cells, and that reactivation of the gene suppresses their growth characteristics. This suggests that inhibin‑α may have a more general tumor suppressor activity outside the reproductive axis.
  

  PMID: 22581369 [PubMed - as supplied by publisher]

• 

  Deficiency in androgens and upregulation of insulin-like growth factor-1 are involved in high bone turnover in men receiving androgen deprivation therapy for prostate cancer.

  Fetched: May 17th, 2012, 11:00pm MDT

  Deficiency in androgens and upregulation of insulin-like growth factor-1 are involved in high bone turnover in men receiving androgen deprivation therapy for prostate cancer.

  Growth Horm IGF Res. 2012 May 11;

  Authors: Ishizaki F, Hara N, Takizawa I, Nishiyama T, Isahaya E, Kawasaki T, Takahashi K

  Abstract
  OBJECTIVE: This study was performed to elucidate the mechanism of high bone turnover during androgen deprivation therapy (ADT) in terms of osteogenic endocrine activity by testosterone, adrenal androgens, and insulin-like growth factor-1 (IGF-I), and to identify markers reflecting the bone mineral density (BMD) during ADT. DESIGN: BMD and samples of blood and urine were studied before and after 6months of ADT in 70 patients with localized prostate cancer. RESULTS: Before ADT, serum free-testosterone, dehydroepiandrosterone sulfate (DHEA-S), androstenedione, and IGF-I levels were correlated with BMD (rs=0.344, p=0.004; rs=0.264, p=0.027; rs=0.329, p=0.005; rs=0.300, p=0.012, respectively). The serum IGF-I level was independently correlated with the pretreatment BMD (Multivariate p=0.001). These relationships disappeared after ADT (p=0.519, 0.316, 0.116, and 0.597, respectively). After ADT, serum levels of free-testosterone decreased (7.9 to 0.2pg/mL), and DHEA-S and androstenedione were also reduced (3.6 to 2.3μmol/L and 5.6 to 2.9nmol/L, respectively) (p<0.001 in all). In contrast, IGF-I levels were elevated after ADT by 11.6% (19.9 to 22.3nmol/L, p<0.001). Delta-values of IGF-I (post- minus pretreatment levels, mean: +2.2, ranged between -7.1 and +15.3) were inversely correlated with the pretreatment (rs=-0.333 p=0.005) and post-treatment (rs=-0.408, p=0.001) BMD. After ADT, the serum IGF-I level was closely correlated with the serum level of the bone formation marker bone-specific alkaline phosphatase (BAP) (rs=0.328, p=0.006), and delta-IGF-I and delta-BAP showed a close positive correlation (rs=0.388, p=0.001). The post-treatment BMD was correlated only with the urine deoxypyridinoline (DPD) concentration (rs=-0.302, p=0.024) among the bone formation/resorption markers including serum/urine N-telopeptide. CONCLUSIONS: Serum IGF-I levels increased during ADT in men with a low BMD. Coupled with reduced androgen levels, elevated IGF-I levels, which were positively correlated with BAP during ADT, possibly explain the mechanism of ADT-related high bone turnover. The increase of IGF-I is more prominent in men whose BMD is already low at the baseline, and urine DPD might be a marker that reflects BMD during ADT.
  

  PMID: 22579549 [PubMed - as supplied by publisher]

• 

  What is your diagnosis? Splenic myelolipoma.

  Fetched: May 17th, 2012, 11:00pm MDT

  What is your diagnosis? Splenic myelolipoma.

  J Am Vet Med Assoc. 2012 Feb 15;240(4):375-6

  Authors: Carrillo JD, Boza S, Soler M, Belda E, Agut A

  PMID: 22309007 [PubMed - indexed for MEDLINE]

• 

  High-throughput molecular and histopathologic profiling of tumor tissue in a novel transplantable model of murine neuroblastoma: new tools for pediatric drug discovery.

  Fetched: May 13th, 2012, 11:00pm MDT

  High-throughput molecular and histopathologic profiling of tumor tissue in a novel transplantable model of murine neuroblastoma: new tools for pediatric drug discovery.

  Cancer Invest. 2012 Jun;30(5):343-63

  Authors: Stauffer JK, Orentas RJ, Lincoln E, Khan T, Salcedo R, Hixon JA, Back TC, Wei JS, Patidar R, Song Y, Hurd L, Tsokos M, Lai EW, Eisenhofer G, Weiss W, Khan J, Wigginton JM

  Abstract
  Using two MYCN transgenic mouse strains, we established 10 transplantable neuroblastoma cell lines via serial orthotopic passage in the adrenal gland. Tissue arrays demonstrate that by histochemistry, vascularity, immunohistochemical staining for neuroblastoma markers, catecholamine analysis, and concurrent cDNA microarray analysis, there is a close correspondence between the transplantable lines and the spontaneous tumors. Several genes closely associated with the pathobiology and immune evasion of neuroblastoma, novel targets that warrant evaluation, and decreased expression of tumor suppressor genes are demonstrated. These studies describe a unique and generalizable approach to expand the utility of transgenic models of spontaneous tumor, providing new tools for preclinical investigation.
  

  PMID: 22571338 [PubMed - in process]

• 

  Persistent hypokalaemia in a Jamaican hypertensive patient.

  Fetched: May 13th, 2012, 11:00pm MDT

  Persistent hypokalaemia in a Jamaican hypertensive patient.

  West Indian Med J. 2011 Dec;60(6):674-7

  Authors: Potu C, Mohammed H, Tulloch-Reid MK

  Abstract
  We report the case of a 48-year old man with uncontrolled hypertension and persistent hypokalaemia from an aldosterone producing adrenal adenoma treated by laparoscopic adrenalectomy. Clinicians' identification of primary hyperaldosteronism is critical as the correct treatment results in improved blood pressure control and reduced risk of complications.
  

  PMID: 22512228 [PubMed - indexed for MEDLINE]

• 

  Cushing's syndrome during pregnancy secondary to adrenal adenoma.

  Fetched: May 13th, 2012, 11:00pm MDT

  Cushing's syndrome during pregnancy secondary to adrenal adenoma.

  Acta Med Iran. 2012;50(1):76-8

  Authors: Borna S, Akbari S, Eftekhar T, Mostaan F

  Abstract
  Pregnancy rarely occurs in untreated cases of Cushing's syndrome (CS) , because most of them are infertile due to significant maternal and fetal complications during pregnancy. Diagnosis of CS may be difficult during pregnancy. Since physiological changes of pregnancy are overlapped by classical presentation and biological confirmation of CS. Therefore the high clinical suspicious is needed for diagnosis. We present a 33 years old pregnant woman with a history of chronic hypertension from 10 years ago that referred to Imam Khomeini hospital for uncontrolled hypertension, gestational diabetes and fetal tachycardia at the 30 weeks of gestation. After initial studies abdominal MRI detected a 43 x 35 x 29 mm right adrenal mass. She was treated by anti-hypertensive drugs. But at 31.5 weeks of gestational age cesarean section was performed due to severe preeclampsia. Then two weeks after delivery open right adrenalectomy was carried out without any complications and in the histopathological evaluation benign adrenocortical adenoma was reported. CS is associated with considerable fetal and maternal morbidity and mortality. Selection of treatment method is variable and it depends on gestational age. Medical and surgical approaches have been used in managing CS in pregnancy. Surgical treatment is the first choice for CS which is recommended at the second trimester and in the late pregnancy medical treatment is preferred.
  

  PMID: 22267384 [PubMed - indexed for MEDLINE]

• 

  Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma.

  Fetched: May 13th, 2012, 11:00pm MDT

  Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma.

  Clin Endocrinol (Oxf). 2011 Nov;75(5):585-91

  Authors: Kroiss M, Quinkler M, Lutz WK, Allolio B, Fassnacht M

  Abstract
  Mitotane [1-(2-chlorophenyl)-1-(4-chlorophenyl)-2,2-dichloroethane, (o,p'-DDD)] is the only drug approved for the treatment for adrenocortical carcinoma (ACC) and has also been used for various forms of glucocorticoid excess. Through still largely unknown mechanisms, mitotane inhibits adrenal steroid synthesis and adrenocortical cell proliferation. Mitotane increases hepatic metabolism of cortisol, and an increased replacement dose of glucocorticoids is standard of care during mitotane treatment. Recently, sunitinib, a multityrosine kinase inhibitor (TKI), has been found to be rapidly metabolized by CYP3A4 during mitotane treatment, indicating clinically relevant drug interactions with mitotane. We here summarize the current evidence concerning mitotane-induced changes in hepatic monooxygenase expression, list drugs potentially affected by mitotane-related CYP3A4 induction and suggest alternatives. For example, using standard doses of macrolide antibiotics is unlikely to reach sufficient plasma levels, making fluoroquinolones in many cases a superior choice. Similarly, statins such as simvastatin are metabolized by CYP3A4, whereas others like pravastatin are not. Importantly, in the past, several clinical trials using cytotoxic drugs but also targeted therapies in ACC yielded disappointing results. This lack of antineoplastic activity may be explained in part by insufficient drug exposure owing to enhanced drug metabolism induced by mitotane. Thus, induction of CYP3A4 by mitotane needs to be considered in the design of future clinical trials in ACC.
  

  PMID: 21883349 [PubMed - indexed for MEDLINE]

• 

  The effect of treatment on monocyte and lymphocyte cytokine release in patients with aldosteronoma.

  Fetched: May 13th, 2012, 11:00pm MDT

  The effect of treatment on monocyte and lymphocyte cytokine release in patients with aldosteronoma.

  Hypertens Res. 2012 Jan;35(1):123-5

  Authors: Krysiak R, Okopien B

  PMID: 21866108 [PubMed - indexed for MEDLINE]

• 

  Retroperitoneal cystic teratoma masquerading as an incidentally discovered adrenal mass.

  Fetched: May 13th, 2012, 11:00pm MDT

  Retroperitoneal cystic teratoma masquerading as an incidentally discovered adrenal mass.

  Endocr Pract. 2011 Sep 1;17(5):e130-4

  Authors: Chen JC, Khiyami A, McHenry CR

  Abstract
  OBJECTIVE: To report a case of a retroperitoneal cystic teratoma that obscured and compressed the adrenal gland, mimicking a primary adrenal tumor.
  METHODS: The presenting manifestations, radiographic characteristics, gross and microscopic pathologic features, and results of surgical therapy and long-term follow-up are described.
  RESULTS: A 50-year-old African American woman with a 2-year history of low back pain and night sweats had a computed tomographic scan of the abdomen, which revealed an incidental 8 by 4 by 3.5-cm left adrenal mass without a clear plane between the mass and the left crus of the diaphragm. Laboratory studies excluded a functioning adrenal tumor. The tumor was resected laparoscopically. It was compressing but not involving the adrenal gland, nor was it involving the diaphragm. Microscopic evaluation revealed a benign mature cystic teratoma characterized by cystic spaces lined by respiratory epithelium with cartilage, bone, lymphoid tissue, smooth muscle, and ganglionic tissue in the cyst wall. The patient had an uneventful postoperative course and is free of recurrence after 18 months of follow-up.
  CONCLUSION: Although rare, a mature cystic teratoma of the retroperitoneum that compresses the normal adrenal gland may masquerade as a primary adrenal tumor and should be included in the differential diagnosis of a nonfunctioning adrenal incidentaloma.
  

  PMID: 21803708 [PubMed - indexed for MEDLINE]

• 

  Performance of plasma fractionated free metanephrines by enzyme immunoassay in the diagnosis of pheochromocytoma and paraganglioma.

  Fetched: May 13th, 2012, 11:00pm MDT

  Performance of plasma fractionated free metanephrines by enzyme immunoassay in the diagnosis of pheochromocytoma and paraganglioma.

  Endocr Pract. 2011 Sep 1;17(5):759-65

  Authors: Sarathi V, Pandit R, Jagtap V, Lila AR, Bandgar TR, Menon PS, Varthakavi P, Raghavan VP, Shah NS

  Abstract
  OBJECTIVE: To study the performance of measuring plasma fractionated free metanephrines by enzyme immunoassay (EIA) in the diagnosis of pheochromocytoma and catecholamine-secreting paraganglioma.
  METHODS: Consecutive patients attending the endocrine clinic at King Edward Memorial Hospital, Mumbai, India, for suspicion of catecholamine-secreting tumors were included. Plasma fractionated free metanephrines were measured by EIA, and computed tomography of the neck, chest, abdomen, and pelvis was performed. Those with tumor identified by imaging underwent 131I m-iodobenzylguanidine scintigraphy. All patients with adrenal masses larger than 3 cm and patients with secretory tumors, irrespective of their size, underwent tumor excision. The rest were followed up for 6 to 12 months.
  RESULTS: One hundred patients with a clinical suspicion of pheochromocytoma or paraganglioma were included. Plasma free normetanephrine alone had a sensitivity of 94.1% (cutoff: 180 ng/mL), while plasma free metanephrine had a sensitivity of 14.7% (cutoff: 90 pg/mL). Both had 96.9% specificity. When combined (either test positive), the sensitivity was 94.1% with a specificity of 93.75%. Thirty-four patients had a histopathologically proven pheochromocytoma or paraganglioma. It was concluded that 66 patients did not harbor a pheochromocytoma or catecholamine-secreting paraganglioma.
  CONCLUSION: Plasma fractionated free metanephrines measured by EIA have good sensitivity and specificity in the diagnosis of pheochromocytoma and catecholamine-secreting paraganglioma.
  

  PMID: 21742610 [PubMed - indexed for MEDLINE]

• 

  Random postoperative day-3 cortisol concentration as a predictor of hypothalamic-pituitary-adrenal axis integrity after transsphenoidal surgery.

  Fetched: May 13th, 2012, 11:00pm MDT

  Random postoperative day-3 cortisol concentration as a predictor of hypothalamic-pituitary-adrenal axis integrity after transsphenoidal surgery.

  Endocr Pract. 2011 Sep 1;17(5):717-26

  Authors: Khan MI, Habra MA, McCutcheon IE, Nogueras-González GM, Devin JK, Busaidy NL, Levine NB, Lindstrom W, Kagan D, Jimenez C, Waguespack SG

  Abstract
  OBJECTIVE: To determine whether a random postoperative day-3 cortisol value of 10 μg/dL or greater is predictive of adrenal sufficiency 3 to 10 weeks after transsphenoidal surgery (TSS) and during long-term clinical follow-up.
  METHODS: We retrospectively reviewed the case records of patients who underwent TSS at our institution between 1991 and 2008. Inclusion criteria were as follows: random cortisol measured on the morning of postoperative day 3, adrenal dynamic testing performed 3 to 10 weeks after TSS, and clinical assessment of the hypothalamic-pituitary-adrenal (HPA) axis at least 6 months after TSS.
  RESULTS: A total of 466 patients underwent TSS at our institution during the study period. Eighty-three patients met study inclusion criteria. Sensitivity of a random postoperative day-3 serum cortisol value of 10 μg/dL or greater for the prediction of adrenal sufficiency at a median follow-up of 42 days was 64.81% (95% confidence interval, 50.6%-77.32%), with an odds ratio of 3.1 (95% confidence interval, 1.08-8.58). Specificity was 62.1% (95% confidence interval, 42.3%-79.3%). At a median follow-up of 500 days, only 2 patients with a postoperative day-3 cortisol value of 10 μg/dL or greater required hydrocortisone replacement, both of whom had multiple anterior pituitary hormone deficiencies and evidence of pituitary dysfunction during the perioperative period.
  CONCLUSIONS: In the appropriate clinical context, a postoperative day-3 cortisol value of 10 μg/dL or greater accurately predicts the integrity of the HPA axis. The final decision regarding corticosteroid replacement should be personalized, considering the postoperative day-3 cortisol level, the clinical context in which the measurement was obtained, and any evidence of concomitant pituitary dysfunction in the perioperative period.
  

  PMID: 21454228 [PubMed - indexed for MEDLINE]

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  Biological tissue diagnostics using needle biopsy and spray ionization mass spectrometry.

  Fetched: May 11th, 2012, 11:00pm MDT

  Biological tissue diagnostics using needle biopsy and spray ionization mass spectrometry.

  Anal Chem. 2011 Dec 15;83(24):9221-5

  Authors: Liu J, Cooks RG, Ouyang Z

  Abstract
  Needle biopsy is a routine medical procedure for examining tissue or biofluids for the presence of disease using standard methods of pathology. In this work, spray ionization directly from tissue in the biopsy needle is shown to provide highly specific molecular information through mass spectrometry analysis. The data are available within a minute after the tissue biopsy, a time scale that allows immediate medical decisions to be made. This method has been performed for tissues in a variety of organs including brain, liver, kidney, adrenal gland, stomach, and spinal cord. Amino acids, hormones, fatty acids, anesthetics, and phospholipids are detected from the tissues and identified using exact mass measurement and tandem mass spectrometry. Lipid profiles are rich in information and, as in imaging MS methods, they have the potential to serve to distinguish diseased from healthy tissue. Needle biopsies allow a crude form of depth profiling that is demonstrated with the analysis of tissue samples taken by a needle inserted into a porcine kidney at various depths.
  

  PMID: 22103750 [PubMed - indexed for MEDLINE]

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  Extravasation of pegylated-liposomal doxorubicin: favorable outcome after immediate subcutaneous administration of corticosteroids.

  Fetched: May 10th, 2012, 11:00pm MDT

  Extravasation of pegylated-liposomal doxorubicin: favorable outcome after immediate subcutaneous administration of corticosteroids.

  Nagoya J Med Sci. 2012 Feb;74(1-2):189-92

  Authors: Mitsuma A, Sawaki M, Shibata T, Morita S, Inada M, Shimokata T, Sugishita M, Kitagawa K, Sawada M, Nawa A, Ando Y

  Abstract
  A massive extravasation of pegylated-liposomal doxorubicin (Doxil) accidentally occurred, affecting the right forearm of a 54-year-old woman with metastatic ovarian cancer who was receiving an intravenous infusion of the drug. In accordance with the institutional guidelines for vesicant drugs, a corticosteroid preparation was immediately injected subcutaneously into the surrounding tissues. Clobetasol propionate and an ice pack were then topically applied to the affected region. There were no serious complications at the extravasation site, such as tissue necrosis or severe pain, and only a transient erythema of the skin and desquamation remained after 2 months.
  

  PMID: 22515126 [PubMed - indexed for MEDLINE]

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  Visinin-like 1 is upregulated in aldosterone-producing adenomas with KCNJ5 mutations and protects from calcium-induced apoptosis.

  Fetched: May 10th, 2012, 11:00pm MDT

  Visinin-like 1 is upregulated in aldosterone-producing adenomas with KCNJ5 mutations and protects from calcium-induced apoptosis.

  Hypertension. 2012 Apr;59(4):833-9

  Authors: Williams TA, Monticone S, Crudo V, Warth R, Veglio F, Mulatero P

  Abstract
  Visinin-like 1 (VSNL1) is upregulated in aldosterone-producing adenomas (APAs) compared with normal adrenals. We demonstrate that VSNL1 overexpression in adrenocortical carcinoma cells (NCI H295R) upregulates basal and angiotensin II-stimulated CYP11B2 gene expression 3.2- and 1.5-fold, respectively. Conversely, silencing VSNL1 by RNA interference decreases angiotensin II-stimulated CYP11B2 expression and aldosterone secretion by 41.0% and 34.5%, respectively. Mutations in the potassium channel KCNJ5 have been identified in APAs that result in sodium influx and membrane depolarization and are postulated to result in calcium influx in adrenal glomerulosa cells. VSNL1 and CYP11B2 are 8.1- and 6.0-fold more highly expressed, respectively, in APAs harboring KCNJ5 mutations compared with those without, and the upregulation of VSNL1 in these APAs accounts for the overexpression of VSNL1 in the total APA sample set compared with normal adrenals. Silencing VSNL1 in H295R cells renders them sensitive to ionomycin-induced apoptosis, indicating that VSNL1 protects these cells against calcium-induced cell death. Concomitant expression of mutated KCNJ5 (G151R) and silencing VSNL1 results in apoptosis of H295R cells, an effect that is blocked by nifedipine and is absent using a control small-interfering RNA or when wild-type KCNJ5 is expressed and VSNL1 is silenced. These data demonstrate that VSNL1 plays a dual function in vitro in the regulation of CYP11B2 gene expression and in the inhibition of calcium-induced apoptosis. In addition, VSNL1 may play a role in the pathophysiology of APAs harboring mutations in the potassium channel KCNJ5 via its antiapoptotic function in response to calcium cytotoxicity and its effect on aldosterone production.
  

  PMID: 22331379 [PubMed - indexed for MEDLINE]

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  The current TNM staging system of renal cell carcinoma: are further improvements needed?

  Fetched: May 10th, 2012, 11:00pm MDT

  The current TNM staging system of renal cell carcinoma: are further improvements needed?

  Arch Esp Urol. 2011 Dec;64(10):929-37

  Authors: Billia M, Volpe A, Terrone C

  Abstract
  Objective of the study is to review the current 7th edition of the TNM classification of renal tumors and to perform a critical analysis of the recent evidence in order to identify the limitations of this new staging system. A search of the english literature was performed through the Medline and Pubmed database using the following keywords: renal cell carcinoma, staging system and TNM. Overall, 2600 references were initially scrutinized. Forty papers were selected based on their pertinence with the topic of the review, level of evidence provided and overall contribution to the field. Few changes have been made in the current version of the TNM staging system of renal tumors. pT2 tumors have been divided in 2 subgroups based on tumor size with a cut-off at 10 cm; the invasion of the renal vein was classified as pT3a; finally, the invasion of the ispsilateral adrenal gland was classified as pT4. However, other changes were suggested by the analysis of the recent literature and have not been introduced in this new version. Further improvements of the TNM classification for renal tumors are needed especially with regard to locally advanced tumors and node-positive disease, in order to improve the accuracy of this important prognostic tool in renal oncology.
  

  PMID: 22228888 [PubMed - indexed for MEDLINE]

• 

  Adalimumab induces and maintains clinical remission in patients with moderate-to-severe ulcerative colitis.

  Fetched: May 10th, 2012, 11:00pm MDT

  Adalimumab induces and maintains clinical remission in patients with moderate-to-severe ulcerative colitis.

  Gastroenterology. 2012 Feb;142(2):257-65.e1-3

  Authors: Sandborn WJ, van Assche G, Reinisch W, Colombel JF, D'Haens G, Wolf DC, Kron M, Tighe MB, Lazar A, Thakkar RB

  Abstract
  BACKGROUND & AIMS: Adalimumab is a fully human monoclonal antibody that binds tumor necrosis factor (TNF)-α. Its efficacy as maintenance therapy for patients with ulcerative colitis has not been studied in a controlled, double-blind trial.
  METHODS: Ulcerative colitis long-term remission and maintenance with adalimumab 2 (ULTRA 2) was a randomized, double-blind, placebo-controlled trial to evaluate the efficacy of adalimumab in induction and maintenance of clinical remission in 494 patients with moderate-to-severe ulcerative colitis who received concurrent treatment with oral corticosteroids or immunosuppressants. Patients were stratified based on prior exposure to TNF-α antagonists (either had or had not been previously treated with anti-TNF-α) and randomly assigned to groups given adalimumab 160 mg at week 0, 80 mg at week 2, and then 40 mg every other week or placebo. Primary end points were remission at weeks 8 and 52.
  RESULTS: Overall rates of clinical remission at week 8 were 16.5% on adalimumab and 9.3% on placebo (P = .019); corresponding values for week 52 were 17.3% and 8.5% (P = .004). Among anti-TNF-α naïve patients, rates of remission at week 8 were 21.3% on adalimumab and 11% on placebo (P = .017); corresponding values for week 52 were 22% and 12.4% (P = .029). Among patients who had previously received anti-TNF agents, rates of remission at week 8 were 9.2% on adalimumab and 6.9% on placebo (P = .559); corresponding values for week 52 were 10.2% and 3% (P = .039). Serious adverse events occurred in 12% of patients given adalimumab or placebo. Serious infections developed in 1.6% of patients given adalimumab and 1.9% given placebo. In the group given adalimumab, 1 patient developed squamous cell carcinoma and 1 developed gastric cancer.
  CONCLUSIONS: Adalimumab was safe and more effective than placebo in inducing and maintaining clinical remission in patients with moderate-to-severe ulcerative colitis who did not have an adequate response to conventional therapy with steroids or immunosuppressants.
  

  PMID: 22062358 [PubMed - indexed for MEDLINE]

• 

  Pheochromocytoma and abdominal paraganglioma.

  Fetched: May 10th, 2012, 11:00pm MDT

  Pheochromocytoma and abdominal paraganglioma.

  J Visc Surg. 2011 Dec;148(6):e409-16

  Authors: Renard J, Clerici T, Licker M, Triponez F

  Abstract
  Pheochromocytomas (PHEO) and paragangliomas (PGL) are tumors derived from the sympathetic and parasympathetic nervous system. The parasympathetic-associated paragangliomas arising in the neck are usually non-functioning and are rarely encountered by general and visceral surgeons. The sympathetic-associated PHEO and PGL are usually functioning and most often arise in the abdomen. Because they harbor very specific characteristics (hypersecretion of catecholamines, familial origin in up to 30% of them, multiple locations, etc.) their perioperative management needs to be known by surgeons taking care of these patients in order to avoid operative disasters. Surgery can lead to perioperative hemodynamic modifications and sometimes catecholamine storm even in normotensive patients with PHEO and PGL. This emphasizes the need to exclude PHEO before any adrenal surgery as well as to medically prepare all patients with PHEO and PGL preoperatively. We review in this paper the pathophysiology and current perioperative management of patients with apparently sporadic PHEO and PGL.
  

  PMID: 21862435 [PubMed - indexed for MEDLINE]

• 

  Metastatic mandibular neuroblastoma: a rare cause of tooth mobility.

  Fetched: May 10th, 2012, 11:00pm MDT

  Metastatic mandibular neuroblastoma: a rare cause of tooth mobility.

  J Clin Pediatr Dent. 2011;36(2):203-6

  Authors: Kürklü E, Emiroğlu HH, Kebudi R, Ozdaş DO, Ayan I, Görgün O, Zülfikar B, Yekeler E, Gülsüm AK

  Abstract
  Neuroblastoma (NBL), a malignant embryonic tumor derived from neural crest cells, is the most common tumor worldwide among children less than 1 year of age. Metastasis to the mandible is uncommon. This article reports the case of a 15-month-old male diagnosed with NBL with bone metastasis including the mandible which resulted in severe tooth mobility. Dentists or pediatricians should consider the primary or metastatic tumors of the maxillofacial region in the differential diagnosis in children presenting with premature loss of teeth related to tooth mobility.
  

  PMID: 22524085 [PubMed - indexed for MEDLINE]

• 

  A rare case of primary hyperparathyroidism associated with primary aldosteronism, hürthle cell thyroid cancer and meningioma.

  Fetched: May 10th, 2012, 11:00pm MDT

  A rare case of primary hyperparathyroidism associated with primary aldosteronism, hürthle cell thyroid cancer and meningioma.

  J Korean Med Sci. 2012 May;27(5):560-4

  Authors: Kim YL, Jang YW, Kim JT, Sung SA, Lee TS, Lee WM, Kim HJ

  Abstract
  Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of endocrine and non-endocrine tumors. There are also a considerable number of atypical MEN1 syndrome. In this case, a 68-yr-old woman was referred to the Department of Endocrinology for hypercalcemia. Five years ago, she had diagnosed as primary hyperaldosteronism and now newly diagnosed as parathyroid hyperplasia with laboratory and pathologic findings. Hürthle-cell thyroid cancer was also resected during the parathyroid exploration and small meningioma was found on brain MRI. Her general condition has markedly improved and her adrenal mass and meningioma are being closely observed now. We could find the loss of heterozygosity of the MEN1 locus in parathyroid glands, suggesting a MEN1-related tumor, but not a germline mutation. Considering a variety of phenotypic expression and a limitation of current molecular analysis, periodic follow up will be needed in patients with a MEN1-like phenotype.
  

  PMID: 22563225 [PubMed - in process]

• 

  Rectal metastases from squamous cell carcinoma: a case report and review of the literature.

  Fetched: May 10th, 2012, 11:00pm MDT

  Rectal metastases from squamous cell carcinoma: a case report and review of the literature.

  Case Report Med. 2012;2012:947524

  Authors: Cedrés S, Mulet-Margalef N, Montero MA, Martinez P, Martínez A, Felip E

  Abstract
  Non-small-cell lung cancer (NSCLC) represents 85% of lung cancer. The most frequent sites of distant metastasis are the liver, adrenal glands, bones and brain. Gastrointestinal metastases are uncommon and rectal metastases are extremely rare. Here we report a case of squamous cell carcinoma of the lung with rectal metastases.
  

  PMID: 22567021 [PubMed - in process]

• 

  High-anxious individuals show increased chronic stress burden, decreased protective immunity, and increased cancer progression in a mouse model of squamous cell carcinoma.

  Fetched: May 6th, 2012, 11:00pm MDT

  High-anxious individuals show increased chronic stress burden, decreased protective immunity, and increased cancer progression in a mouse model of squamous cell carcinoma.

  PLoS One. 2012;7(4):e33069

  Authors: Dhabhar FS, Saul AN, Holmes TH, Daugherty C, Neri E, Tillie JM, Kusewitt D, Oberyszyn TM

  Abstract
  In spite of widespread anecdotal and scientific evidence much remains to be understood about the long-suspected connection between psychological factors and susceptibility to cancer. The skin is the most common site of cancer, accounting for nearly half of all cancers in the US, with approximately 2-3 million cases of non-melanoma cancers occurring each year worldwide. We hypothesized that a high-anxious, stress-prone behavioral phenotype would result in a higher chronic stress burden, lower protective-immunity, and increased progression of the immuno-responsive skin cancer, squamous cell carcinoma. SKH1 mice were phenotyped as high- or low-anxious at baseline, and subsequently exposed to ultraviolet-B light (1 minimal erythemal dose (MED), 3 times/week, 10-weeks). The significant strengths of this cancer model are that it uses a normal, immunocompetent, outbred strain, without surgery/injection of exogenous tumor cells/cell lines, and produces lesions that resemble human tumors. Tumors were counted weekly (primary outcome), and tissues collected during early and late phases of tumor development. Chemokine/cytokine gene-expression was quantified by PCR, tumor-infiltrating helper (Th), cytolytic (CTL), and regulatory (Treg) T cells by immunohistochemistry, lymph node T and B cells by flow cytometry, adrenal and plasma corticosterone and tissue vascular-endothelial-growth-factor (VEGF) by ELISA. High-anxious mice showed a higher tumor burden during all phases of tumor development. They also showed: higher corticosterone levels (indicating greater chronic stress burden), increased CCL22 expression and Treg infiltration (increased tumor-recruited immuno-suppression), lower CTACK/CCL27, IL-12, and IFN-γ gene-expression and lower numbers of tumor infiltrating Th and CTLs (suppressed protective immunity), and higher VEGF concentrations (increased tumor angiogenesis/invasion/metastasis). These results suggest that the deleterious effects of high trait anxiety could be: exacerbated by life-stressors, accentuated by the stress of cancer diagnosis/treatment, and mediate increased tumor progression and/or metastasis. Therefore, it may be beneficial to investigate the use of chemotherapy-compatible anxiolytic treatments immediately following cancer diagnosis, and during cancer treatment/survivorship.
  

  PMID: 22558071 [PubMed - in process]

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  Mining the proteome - the application of tandem mass spectrometry to endocrine cancer research.

  Fetched: May 6th, 2012, 11:00pm MDT

  Mining the proteome - the application of tandem mass spectrometry to endocrine cancer research.

  Endocr Relat Cancer. 2012 May 3;

  Authors: Sharma N, Martin A, McCabe CJ

  Abstract
  Tandem mass spectrometry (MS/MS) is at the forefront of proteome based cancer research, permitting the detection of femtomolar amounts of protein from a wide variety of tissue sources. As endocrine cancers are frequently aetiologically complex, they are particularly amenable to mass spectrometry. The most widely studied aspect is the search for novel, reliable biomarkers which will allow cancers to be diagnosed earlier and distinguished from benign tumours. MS/MS allows for the rapid analysis of blood and urine in addition to tumour tissue, and in this regard has been applied to research involving thyroid, pancreatic, adrenal and ovarian cancer with varying degrees of success. The description of an individual cancer proteome potentially allows for personalised management of each patient, avoiding unnecessary therapies and targeting treatments to those which will have the most effect. The application of MS/MS to interaction proteomics is a field that has generated recent novel targets for chemotherapy. However, the technology involved in MS/MS has a number of drawbacks that at present prevent its widespread use in translational cancer research. There is poor reproducibility of results, in part due to the large amount of data generated and the inability to accurately differentiate true from false positive results. Further, current cost of running MS/MS restricts the number of times experiments can be repeated, contributing to the lack of significance and concordance between studies. Despite these problems, however, MS/MS is emerging as a front line tool in endocrine cancer research and it is likely that this will continue over the next decade.
  

  PMID: 22555494 [PubMed - as supplied by publisher]

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  Prognostic factors in renal cell carcinoma: analysis of 227 patients treated at the Brazilian National Cancer Institute.

  Fetched: May 6th, 2012, 11:00pm MDT

  Prognostic factors in renal cell carcinoma: analysis of 227 patients treated at the Brazilian National Cancer Institute.

  Int Braz J Urol. 2012 Mar;38(2):185-94

  Authors: Ornellas AA, Andrade DM, Ornellas P, Wisnescky A, Schwindt AB

  Abstract
  Purpose: We evaluated the role of several prognostic factors in predicting death and/or progressive disease in patients with renal cell carcinoma. Materials and Methods: Between 2004 and 2010, 227 consecutive patients with renal cell carcinoma underwent radical nephrectomy at our Institute. All histological specimens were examined by the same pathologist. We considered certain histological parameters, including histological subtype, conventional Fuhrman grade, presence of sarcomatoid features, adrenal gland infi ltration, invasion of the perinephric fat, vascular embolization, collecting system invasion, presence or absence of tumour necrosis (0 %, 1 % to 49 %, or 50 % or greater) and regional lymph node metastasis. Results: Variables signifi cantly associated with death and/or progressive disease on univariate analysis were histological subtype (p = 0.006), Fuhrman grade (p < 0.0001), tumor necrosis (p = 0.009), perinephric fat invasion (p = 0.002), vascular embolization (p = 0.0002), presence of lymph node involvement (p < 0.002), tumor size (p = 0.0006), TNM stage (p < 0.00001) and presence of metastasis (p < 0.00001). In the multivariable model histological subtype, tumor necrosis, lymph node involvement and presence of metastasis were independent risk factors for disease-free survival (p = 0.011, 0.042, 0.025 and p < 0.0001, respectively). Conclusion: Histological subtype, tumor necrosis, lymph node involvement and presence of metastasis proved to be independent prognostic factors for disease-free survival. Therefore, the presence and rate of tumor necrosis should always be informed by the pathologist and lymphadenectomy should be performed in all patients.
  

  PMID: 22555027 [PubMed - in process]

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  Robotic posterior retroperitoneal adrenalectomy: for what benefit and at what cost?: Comment on "Robotic vs laparoscopic posterior retroperitoneal adrenalectomy".

  Fetched: May 6th, 2012, 11:00pm MDT

  Robotic posterior retroperitoneal adrenalectomy: for what benefit and at what cost?: Comment on "Robotic vs laparoscopic posterior retroperitoneal adrenalectomy".

  Arch Surg. 2012 Mar;147(3):275-6

  Authors: Kebebew E

  PMID: 22430912 [PubMed - indexed for MEDLINE]

• 

  Robotic vs laparoscopic posterior retroperitoneal adrenalectomy.

  Fetched: May 6th, 2012, 11:00pm MDT

  Robotic vs laparoscopic posterior retroperitoneal adrenalectomy.

  Arch Surg. 2012 Mar;147(3):272-5

  Authors: Agcaoglu O, Aliyev S, Karabulut K, Siperstein A, Berber E

  Abstract
  OBJECTIVE: To compare robotic vs laparoscopic posterior retroperitoneal adrenalectomy with regard to perioperative outcomes.
  DESIGN: Prospectively study.
  SETTING: Tertiary academic center.
  PATIENTS: Thirty-one patients who underwent robotic posterior retroperitoneal adrenalectomy and 31 consecutive patients who underwent laparoscopic posterior retroperitoneal adrenalectomy from a prospective institutional review board-approved database.
  MAIN OUTCOME MEASURES: Demographic and clinical parameters, operative time, presence of complications, length of hospital stay, and pain score on postoperative days 1 and 14.
  RESULTS: The mean (SEM) tumor sizes for the robotic and laparoscopic groups were similar (3.1 [0.2] and 3.0 [0.2] cm, respectively; P = .48). For all patients, the mean (SEM) skin-to-skin operative times were similar in both groups (163.2 [10.1] and 165.7 [9.5] minutes, respectively; P = .43). When the last 21 patients who underwent robotic posterior retroperitoneal adrenalectomy were compared with the 31 patients from the laparoscopic series, it was seen that the mean (SEM) operative time was shorter for the robotic group than for the laparoscopic group (139.1 [10.9] vs 166.9 [8.2] minutes; P = .046). The mean (SEM) estimated blood losses and hospital stays were similar between groups. The mean (SEM) pain score on postoperative day 1 was lower in the robotic group than in the laparoscopic group (2.5 [0.3] vs 4.2 [0.4]; P = .008); however, the mean (SEM) pain scores for the groups were similar on postoperative day 14 (P = .53). There were no deaths or cases of morbidity in either group.
  CONCLUSIONS: Our study shows that, beyond the learning curve for experienced laparoscopic surgeons, robotic posterior retroperitoneal adrenalectomy shortens the skin-to-skin operative time compared with the laparoscopic approach. Our results also suggest that the immediate postoperative pain may be less severe for patients who undergo robotic posterior retroperitoneal adrenalectomy.
  

  PMID: 22430911 [PubMed - indexed for MEDLINE]

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  A case of primary retroperitoneal teratoma presenting as an adrenal incidentaloma.

  Fetched: May 6th, 2012, 11:00pm MDT

  A case of primary retroperitoneal teratoma presenting as an adrenal incidentaloma.

  J Endocrinol Invest. 2011 Sep;34(8):645-6

  Authors: Giordano R, Giraudo G, Forno D, Bosco M, Delsedime L, Morino M, Arvat E

  PMID: 22080648 [PubMed - indexed for MEDLINE]

• 

  Mixed Corticomedullary Carcinoma of the Adrenal Gland: A Case Report.

  Fetched: May 5th, 2012, 11:00pm MDT

  Mixed Corticomedullary Carcinoma of the Adrenal Gland: A Case Report.

  Endocr Pract. 2012 May 1;:1-17

  Authors: Turk AT, Asad H, Trapasso J, Perilli G, Livolsi VA

  Abstract
  Objective: To report the case of a 78-year-old woman with mixed corticomedullary carcinoma of the adrenal gland, and review other lesions that exhibit clinical and/or histopathologic features of both adrenal cortical and medullary differentiation.Methods: We describe the patient's clinical case and laboratory test results, as well as the gross and histopathologic features of her tumor. We also review the literature pertaining to mixed corticomedullary adenomas, as well as adrenal cortical tumors with clinical features of pheochromocytoma, and vice versa.Results: A 78-year-old woman with a 10-cm left adrenal mass was hospitalized for management of hypertensive urgency. Laboratory work-up revealed elevated urinary metanephrine levels, as well as elevated serum dehydroepiandrosterone sulfate (DHEAS). She underwent left adrenalectomy. Pathologic examination of the lesion showed mixed cortical and medullary histology, as well as gross and microscopic evidence of malignancy. Including the present case, we identified 17 cases of neoplasms that exhibit features of mixed corticomedullary differentiation, among which this case represents the first malignant tumor.Conclusion: We believe this report represents the first documented case of mixed corticomedullary carcinoma. Several benign lesions combine clinical, biochemical, and/or histopathologic evidence of both adrenal cortical and medullary differentiation, including mixed corticomedullary adenomas, ACTH-secreting pheochromocytomas, et cetera. This report indicates that the differential diagnosis of a lesion with mixed cortical and medullary features should also include a malignant neoplasm.
  

  PMID: 22548942 [PubMed - as supplied by publisher]

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  Multicentric neurofibromatosis with rectal prolapse in a California sea lion (Zalophus californianus).

  Fetched: May 5th, 2012, 11:00pm MDT

  Multicentric neurofibromatosis with rectal prolapse in a California sea lion (Zalophus californianus).

  J Zoo Wildl Med. 2012 Mar;43(1):110-9

  Authors: Rush EM, Ogburn AL, Garner MM

  Abstract
  An approximately 31-yr-old California sea lion (Zalophus californianus) with a history of chronic visual impairment and corneal disease presented with slow onset, progressive neurologic deficits. Treatment for rear flipper paresis was not effective and the animal was euthanatized. Histopathologic findings included hepatocellular and biliary neoplasia, ocular amyloidosis, adrenal adenoma and pheochromocytoma, and spinal cord changes consistent with multicentric neurofibromatosis. This is the first documentation of these conditions in a California sea lion.
  

  PMID: 22448517 [PubMed - indexed for MEDLINE]

• 

  Mitotane exhibits dual effects on steroidogenic enzymes gene transcription under basal and cAMP-stimulating microenvironments in NCI-H295 cells.

  Fetched: May 3rd, 2012, 11:00pm MDT

  Mitotane exhibits dual effects on steroidogenic enzymes gene transcription under basal and cAMP-stimulating microenvironments in NCI-H295 cells.

  Toxicology. 2012 Apr 23;

  Authors: Lin CW, Chang YH, Pu HF

  Abstract
  Adrenocortical carcinoma (ACC) is an extremely rare and aggressive endocrine malignancy with a poor prognosis. The most common symptom of ACC is hypercortisolism (Cushing's syndrome), which has the highest mortality. Mitotane is used as a steroidogenesis inhibitor for Cushing's syndrome or as a chemical adrenalectomy drug for ACC. Mitotane induces adrenal cortex necrosis, mitochondrial membrane impairment, and irreversible binding to CYP proteins. In this study, we explored the molecular effect of mitotane on steroidogenesis in human adrenocortical cancer NCI-H295 cells. Mitotane (10-40μM) inhibited basal and cAMP-induced cortisol secretion but did not cause cell death. Mitotane exhibited an inhibitory effect on the basal expression of StAR and P450scc protein. Furthermore, 40μM of mitotane significantly diminished StAR, CYP11A1 and CYP21 mRNA expression. HSD3B2 and CYP17 seem to be insensitive to mitotane. The stimulatory effects of mitotane on CYP11B1 were more remarkable than its inhibitory effects. In contrast, the activation of cAMP signaling strongly elevated the expression of all these genes. Mitotane (40μM) almost completely neutralized this positive effect and returned 8-Br-cAMP-induced StAR, CYP11A1, CYP17 and CYP21 mRNA to control levels. After cAMP activation, mitotane did not change the levels of CYP11B1 mRNA. The present study demonstrates that mitotane can inhibit cortisol biosynthesis due to a non-specific interference with the gene transcription of steroidogenic enzymes under both basal and 8-Br-cAMP-activated conditions in NCI-H295 cells. We also identified that StAR and CYP11A1 key enzymes that participate in the rate-limiting step of steroidogenesis, were more sensitive to mitotane. In addition, the biphasic effect of mitotane on CYP11B1 was also elucidated.
  

  PMID: 22546480 [PubMed - as supplied by publisher]

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  Cancer detection and biopsy classification using concurrent histopathological and metabolomic analysis of core biopsies.

  Fetched: May 3rd, 2012, 11:00pm MDT

  Cancer detection and biopsy classification using concurrent histopathological and metabolomic analysis of core biopsies.

  Genome Med. 2012 Apr 30;4(4):33

  Authors: Brown MV, McDunn JE, Gunst PR, Smith EM, Milburn MV, Troyer DA, Lawton KA

  Abstract
  ABSTRACT: BACKGROUND: Metabolomics, the non-targeted interrogation of small molecules in a biological sample, is an ideal technology for identifying diagnostic biomarkers. Current tissue extraction protocols involve sample destruction, precluding additional uses of the tissue. This is particularly problematic for high value samples with limited availability, such as clinical tumor biopsies that require structural preservation to histologically diagnose and gauge cancer aggressiveness. To overcome this limitation and increase the amount of information obtained from patient biopsies, we developed and characterized a workflow to perform metabolomic analysis and histological evaluation on the same biopsy sample. METHODS: Biopsies of ten human tissues (muscle, adrenal gland, colon, lung, pancreas, small intestine, spleen, stomach, prostate, kidney) were placed directly in a methanol solution to recover metabolites, precipitate proteins, and fix tissue. Following incubation, biopsies were removed from the solution and processed for histology. Kidney and prostate cancer tumor and benign biopsies were stained with hemotoxylin and eosin and prostate biopsies were subjected to PIN-4 immunohistochemistry. The methanolic extracts were analyzed for metabolites on GC/MS and LC/MS platforms. Raw mass spectrometry data files were automatically extracted using an informatics system that includes peak identification and metabolite identification software. RESULTS: Metabolites across all major biochemical classes (amino acids, peptides, carbohydrates, lipids, nucleotides, cofactors, xenobiotics) were measured. The number (ranging from 260 in prostate to 340 in colon) and identity of metabolites were comparable to results obtained with the current method requiring 30 mg ground tissue. Comparing relative levels of metabolites, cancer tumor from benign kidney and prostate biopsies could be distinguished. Successful histopathological analysis of biopsies by chemical staining (hematoxylin, eosin) and antibody binding (PIN-4, in prostate) showed cellular architecture and immunoreactivity were retained. CONCLUSIONS: Concurrent metabolite extraction and histological analysis of intact biopsies is amenable to the clinical workflow. Methanol fixation effectively preserves a wide range of tissues and is compatible with chemical staining and immunohistochemistry. The method offers an opportunity to augment histopathological diagnosis and tumor classification with quantitative measures of biochemicals in the same tissue sample. Since certain biochemicals have been shown to correlate with disease aggressiveness, this method should prove valuable as an adjunct to differentiate cancer aggressiveness.
  

  PMID: 22546470 [PubMed - as supplied by publisher]

• 

  Recurrent ventricular tachycardia in malignant metastatic pheochromocytoma.

  Fetched: May 3rd, 2012, 11:00pm MDT

  Recurrent ventricular tachycardia in malignant metastatic pheochromocytoma.

  Circulation. 2012 Mar 13;125(10):e435-8

  Authors: Park JW, Park SJ, Hur KY, Kim JH, Choi YL, Park SM, Kim SM, Koo EH, Kim JS

  PMID: 22412096 [PubMed - indexed for MEDLINE]

• 

  Clinical characteristics of patients with remitting seronegative symmetrical synovitis with pitting edema compared to patients with pure polymyalgia rheumatica.

  Fetched: May 3rd, 2012, 11:00pm MDT

  Clinical characteristics of patients with remitting seronegative symmetrical synovitis with pitting edema compared to patients with pure polymyalgia rheumatica.

  J Rheumatol. 2012 Jan;39(1):148-53

  Authors: Kimura M, Tokuda Y, Oshiawa H, Yoshida K, Utsunomiya M, Kobayashi T, Deshpande GA, Matsui K, Kishimoto M

  Abstract
  OBJECTIVE: To compare clinical features of patients with remitting seronegative symmetrical synovitis with pitting edema (RS3PE) and patients with polymyalgia rheumatica (PMR) and to explore the purported association between RS3PE and malignancy.
  METHODS: We did a retrospective chart review of patients with RS3PE and PMR treated in a community-based hospital between January 2000 and December 2009. Outcomes assessed were clinical course of disease and associated malignancies.
  RESULTS: We identified 28 patients with RS3PE and 123 with pure PMR. All patients with RS3PE fulfilled PMR criteria as well. Age, comorbidity, erythrocyte sedimentation rate, duration and progression of symptoms, treatment response to initial low-dose steroids, and steroid complication rates were similar in both groups. Patients with RS3PE were more likely to be male (79% vs 41%; p = 0.001) and to have a history of smoking (39% vs 15%; p = 0.008) and a higher rate of depression (11% vs 2%; p = 0.044) at diagnosis. Among those with RS3PE, hip pain was less common (39% vs 74%; p = 0.001) than in the PMR group. No patients with RS3PE and 6 patients with pure PMR (4.9%) developed another rheumatological disease during followup. Seven of 9 patients (78%) with concurrent cancer presented slightly more frequently with systemic symptoms compared to patients without cancer (48%; p = 0.098), especially with fatigue (56% vs 22%; p = 0.037) and anorexia (33% vs 9.0%; p = 0.047). Despite rigorous cancer screening in patients with RS3PE, however, the rate of associated malignancy was not statistically different from that of patients with pure PMR [2 (7%) vs 7 (6%), respectively; p = 0.673].
  CONCLUSION: Despite evidence that RS3PE is clinically distinct from PMR, we observed characteristics, treatment response, and outcomes like those expected in pure PMR. Compared to patients with pure PMR, patients with RS3PE are more likely to be male, to be depressed, and to smoke. Contrary to earlier studies, no clear association of RS3PE with malignancy was found despite rigorous cancer screening, although clinicians should be aware that patients with concurrent cancer may manifest more systemic signs and symptoms, as well as steroid resistance.
  

  PMID: 22174210 [PubMed - indexed for MEDLINE]

• 

  Metastatic clear cell renal cell carcinoma to the adrenal gland without an identifiable primary tumor.

  Fetched: May 3rd, 2012, 11:00pm MDT

  Metastatic clear cell renal cell carcinoma to the adrenal gland without an identifiable primary tumor.

  Int J Urol. 2012 Jan;19(1):92-3

  Authors: Johnson MT, Bahnson RR, Zynger DL

  PMID: 22146098 [PubMed - indexed for MEDLINE]

• 

  [Role of endoscopic adrenalectomy in adrenal gland surgery].

  Fetched: May 3rd, 2012, 11:00pm MDT

  [Role of endoscopic adrenalectomy in adrenal gland surgery].

  Zentralbl Chir. 1998;123(5):495-500

  Authors: Heintz A, Junginger T

  Abstract
  Endoscopic techniques are used increasingly in adrenal gland surgery. In this paper the indications and results of the laparoscopic and retroperitoneoscopic approach are compared to the results of the conventional trans- and extraperitoneal approach. Endoscopic adrenalectomy can be performed safely and with the benefits associated with minimally invasive surgery. Disadvantages of the laparoscopic, transperitoneal approach are the risk of an intraabdominal injury and the problems caused by adhesions after abdominal operations. The retroperitoneoscopic approach avoids these disadvantages. Anatomical orientation is more difficult using the retroperitoneoscopic access. In case of large tumors of the adrenal gland suspected for malignancy we see the indication for the conventional transperitoneal approach.
  

  PMID: 22462217 [PubMed - indexed for MEDLINE]

• 

  Three uncommon adrenal incidentalomas: a 13-year surgical pathology review.

  Fetched: May 2nd, 2012, 11:00pm MDT

  Three uncommon adrenal incidentalomas: a 13-year surgical pathology review.

  World J Surg Oncol. 2012 Apr 27;10(1):64

  Authors: Kanthan R, Senger JL, Kanthan SC

  Abstract
  ABSTRACT: BACKGROUND: The discovery of adrenal incidentalomas due to the widespread use of sophisticated abdominal imaging techniques has resulted in an increasing trend of adrenal gland specimens being received in the pathology laboratory. In this context, we encountered three uncommon adrenal incidentalomas. The aim of this manuscript is to report in detail the three index cases of adrenal incidentalomas in the context of a 13-year retrospective surgical pathology review. METHODS: The three index cases were investigated and analyzed in detail with relevant review of the English literature as available in PubMed and Medline. A 13-year retrospective computer-based histopathological surgical review was conducted in our laboratory and the results were analyzed in the context of evidence-based literature on adrenal incidentalomas. RESULTS: A total of 94 adrenal specimens from incidentalomas were identified, accounting for 0.025% of all surgical pathology cases. In all 76.6% were benign and 23.4% were malignant. A total of 53 females (56.4%) and 41 males (43.6%) aged 4 to 85 years were identified. The benign lesions included cortical adenoma (43.1%), pheochromocytoma (29.3%) and inflammation/fibrosis/hemorrhage (8.3%). Metastatic neoplasms were the most common malignant lesions (50%) followed by primary adrenocortical carcinomas (31.8%) and neuroblastoma (13.6%). These cases were discovered as adrenal incidentalomas that led to surgical exploration.The three index cases of adrenal incidentalomas with unusual pathologies were encountered that included (a) adrenal ganglioneuroma, (b) periadrenal schwannoma and (c) primary adrenal pleomorphic leiomyosarcoma. These cases are discussed, with a literature and clinicopathological review. CONCLUSIONS: Adrenal lesions are uncommon surgical specimens in the pathology laboratory. However, higher detection rates of adrenal incidentalomas aided by the ease of laparoscopic adrenalectomy has resulted in increased adrenal surgical specimens leading to unsuspected diagnostic and management dilemmas. Accurate pathological identification of common and uncommon adrenal incidentalomas is essential for optimal patient management.
  

  PMID: 22540324 [PubMed - as supplied by publisher]

• 

  Transarterial embolization and doxorubicin eluting beads-transarterial chemoembolization (DEB-TACE) of malignant extra-adrenal pheochromocytoma.

  Fetched: April 30th, 2012, 11:00pm MDT

  Transarterial embolization and doxorubicin eluting beads-transarterial chemoembolization (DEB-TACE) of malignant extra-adrenal pheochromocytoma.

  Cardiovasc Intervent Radiol. 2011 Dec;34(6):1325-9

  Authors: Kumar P, Bryant T, Breen D, Stedman B, Hacking N

  PMID: 21437761 [PubMed - indexed for MEDLINE]

• 

  [Adrenal oncocytoma].

  Fetched: April 30th, 2012, 11:00pm MDT

  [Adrenal oncocytoma].

  Cir Esp. 2011 Dec;89(10):692-3

  Authors: Reyna-Villasmil E, Santos-Bolívar J, Prieto-Montaño J

  PMID: 21419397 [PubMed - indexed for MEDLINE]

• 

  [Adrenal extramedullary plasmacytoma].

  Fetched: April 30th, 2012, 11:00pm MDT

  [Adrenal extramedullary plasmacytoma].

  Cir Esp. 2011 Dec;89(10):690-1

  Authors: Blanco Antona F, Bahamonde Cabria S, Blanco Antona L, Marín Pérez-Tabernero A

  PMID: 21414618 [PubMed - indexed for MEDLINE]

• 

  Traditional and novel cardiovascular risk factors in non-functioning adrenal adenomas.

  Fetched: April 30th, 2012, 11:00pm MDT

  Traditional and novel cardiovascular risk factors in non-functioning adrenal adenomas.

  Eur J Intern Med. 2012 Jan;23(1):83-7

  Authors: Yener S, Cömlekci A, Yuksel F, Sevinc A, Ertilav S, Yesil S

  Abstract
  BACKGROUND: The majority of the incidentally discovered adrenal masses are non-functioning adrenal adenomas; however data regarding traditional and novel cardiovascular risk predictors in these subjects is lacking. The objective of our study was to investigate the levels of PAI-1, IL-6 and Apelin along with several traditional cardiovascular risk markers in subjects with non-functioning adrenal adenomas.
  METHODS: 38 subjects with non-functioning adrenal adenomas and 30, age, gender and BMI matched healthy controls were enrolled. Participants underwent hormonal evaluation including morning cortisol, adrenocorticotrophic hormone (ACTH), post dexamethasone suppression test (DST) cortisol, dehydroepiandrosterone sulfate (DHEAS) and urinary cortisol. Anthropometric and metabolic parameters, body composition, PAI-1, IL-6 and Apelin were measured.
  RESULTS: Subjects with non-functioning adrenal adenomas had significant elevations in systolic blood pressure, mean arterial pressure, waist circumference, uric acid, and post DST cortisol and had significantly reduced levels of DHEAS when compared to BMI matched controls. No significant difference was observed in terms of PAI-1, IL-6 and Apelin between groups. PAI-1 and IL-6 were significantly correlated with mean arterial pressure, BMI, uric acid, total and LDL-cholesterol. Linear regression analysis showed that morning cortisol and Apelin levels independently predicted HOMA levels in subjects with adrenal adenomas.
  CONCLUSION: Subjects with non-functioning adrenal adenomas feature several cardiovascular risk factors even when compared to BMI matched individuals. Subtle cortisol autonomy in adrenal adenomas may be associated with those findings.
  

  PMID: 22153537 [PubMed - indexed for MEDLINE]

• 

  Chronic obstructive pulmonary disease.

  Fetched: April 30th, 2012, 11:00pm MDT

  Chronic obstructive pulmonary disease.

  Lancet. 2012 Apr 7;379(9823):1341-51

  Authors: Decramer M, Janssens W, Miravitlles M

  Abstract
  Chronic obstructive pulmonary disease (COPD) is characterised by progressive airflow obstruction that is only partly reversible, inflammation in the airways, and systemic effects or comorbities. The main cause is smoking tobacco, but other factors have been identified. Several pathobiological processes interact on a complex background of genetic determinants, lung growth, and environmental stimuli. The disease is further aggravated by exacerbations, particularly in patients with severe disease, up to 78% of which are due to bacterial infections, viral infections, or both. Comorbidities include ischaemic heart disease, diabetes, and lung cancer. Bronchodilators constitute the mainstay of treatment: β(2) agonists and long-acting anticholinergic agents are frequently used (the former often with inhaled corticosteroids). Besides improving symptoms, these treatments are also thought to lead to some degree of disease modification. Future research should be directed towards the development of agents that notably affect the course of disease.
  

  PMID: 22314182 [PubMed - indexed for MEDLINE]

• 

  Laparoscopic adrenalectomy for functioning and non-functioning adrenal tumours.

  Fetched: April 28th, 2012, 11:00pm MDT

  Laparoscopic adrenalectomy for functioning and non-functioning adrenal tumours.

  Endokrynol Pol. 2011;62(6):512-6

  Authors: Lubikowski J, Kiedrowicz B, Szajko M, Andrysiak-Mamos E, Pynka S, Wójcicki M, Jarosz K, Koziołek M, Fuchs H, Post M, Safranow K, Syrenicz A

  Abstract
  BACKGROUND: The purpose of this study was a retrospective analysis of outcomes following laparoscopic adrenalectomy (LA) performed for benign adrenal tumours responsible for various endocrinological disorders. The patients were diagnosed with non-functioning (NFT) and functioning adrenal tumours (FT) including pheochromocytoma (PH), Conn's syndrome (CO) and Cushing's (CS) syndrome.
  MATERIAL AND METHODS: A total of 165 LAs were carried out between August 1995 and September 2009 via either the transperitoneal (n = 38) or retroperitoneal (n = 127) approach. The analysed factors included demographic data of patients, the American Association of Anaesthesiology score (ASA), indication for surgery, tumour size and side, intraoperative and postoperative outcome of LA including duration of surgery, blood loss, time until ambulation, length of hospital stay, time until return to normal activity, the complication rate, as well as the conversion rate to open adrenalectomy.
  RESULTS: There were 111 patients with NFT and 54 with FT. Patients with NFT were significantly older than those with CO (p < 0.05). The mean size of the lesion differed between CO and other adrenal tumours (p < 0.05) as well as between NFT and PH (p < 0.05). All the lesions except aldosteronomas were detected predominantly in the right adrenal gland (p < 0.05). However, despite the different characteristic and clinical disorders related to laparoscopically removed adrenal tumours, the intraoperative and postoperative outcomes did not significantly differ in most cases between the analysed groups of patients.
  CONCLUSION: This study shows that LA is a safe, effective, and well-tolerated procedure despite the hormonal activity of the removed lesions. Minimal invasive surgery may be recommended as the 'gold standard' in the treatment of both functioning and non-functioning benign tumours of the adrenal gland.
  

  PMID: 22144217 [PubMed - indexed for MEDLINE]

• 

  Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011?

  Fetched: April 27th, 2012, 11:00pm MDT

  Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011?

  Horm Cancer. 2011 Dec;2(6):363-71

  Authors: Baudin E, Leboulleux S, Al Ghuzlan A, Chougnet C, Young J, Deandreis D, Dumont F, Dechamps F, Caramella C, Chanson P, Lanoy E, Borget I, Schlumberger M

  Abstract
  The prognosis of advanced adrenocortical carcinoma (ACC) is dismal but heterogeneous. In 2011, mitotane is the only drug approved in Europe and US for the treatment of advanced ACC. Mitotane exerts both antisecretory and antiproliferative effects, which are delayed over time, and requires careful biological and morphological evaluations coupled with mitotane plasma measurement monitoring. In the absence of demonstration of any superior activity of combined polychemotherapy, the least toxic regimen should be considered in routine care. Locoregional therapies, including surgery of the primary tumor and metastases, should be considered part of the therapeutic arsenal. A prolonged survival can be observed in the case of tumor objective response and/or high plasma mitotane levels. New protocols are urgently needed, coupled with ancillary studies dedicated to progress in the findings of predictors or surrogates. International networks and comprehensive databases gathering clinical and biological data constitute the prerequisites for progress.
  

  PMID: 22161625 [PubMed - indexed for MEDLINE]

• 

  Eplerenone use in primary aldosteronism during pregnancy.

  Fetched: April 27th, 2012, 11:00pm MDT

  Eplerenone use in primary aldosteronism during pregnancy.

  Hypertension. 2012 Feb;59(2):e18-9

  Authors: Cabassi A, Rocco R, Berretta R, Regolisti G, Bacchi-Modena A

  PMID: 22146514 [PubMed - indexed for MEDLINE]

• 

  FDG PET in the management of patients with adrenal masses and adrenocortical carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  FDG PET in the management of patients with adrenal masses and adrenocortical carcinoma.

  Horm Cancer. 2011 Dec;2(6):354-62

  Authors: Deandreis D, Leboulleux S, Caramella C, Schlumberger M, Baudin E

  Abstract
  Adrenocortical carcinoma (ACC) is a rare tumor with aggressive behavior, high recurrence rate, and rapid evolution. Surgery is the only curative modality, while systemic treatments such as mitotane and chemotherapy associated to locoregional therapeutic tools remain as palliative options. Imaging has an important role in the management of patients with ACC both at diagnosis and during follow-up. First, it is necessary to characterize undetermined adrenal masses, selecting patients for surgery. Then, in case of malignancy, it is mandatory to assess disease extension, to detect early relapse during follow-up, and to evaluate treatment response. Computed tomography scan and magnetic resonance imaging are actually the most used techniques for these intents as they are widely available in clinical practice. (18)F-fluorodeoxyglucose positron emission tomography (FDG PET) is routinely used for other malignancies and, on the basis of published data, is also becoming a promising tool in the management of ACC. Not only is it a diagnostic tool complementary to morphological imaging in the characterization of adrenal masses and in tumoral lesions detection, but it can be also useful to evaluate tumor response to treatment. New tracers and indications for the clinical use of FDG PET in this specific disease still have to be evaluated to assess its role in clinical practice.
  

  PMID: 22076881 [PubMed - indexed for MEDLINE]

• 

  Conventional imaging in adrenocortical carcinoma: update and perspectives.

  Fetched: April 27th, 2012, 11:00pm MDT

  Conventional imaging in adrenocortical carcinoma: update and perspectives.

  Horm Cancer. 2011 Dec;2(6):341-7

  Authors: Young WF

  Abstract
  The objectives of this study were to review how conventional imaging for adrenocortical carcinoma has evolved over the past 100 years and to highlight the current role for computed tomography (CT) and magnetic resonance imaging (MRI). Using historical cases from the Mayo Clinic archives, the approaches to conventional imaging for adrenocortical carcinoma are described, and pertinent literature is reviewed. Limited conventional imaging options in the first 75 years of the twentieth century were supplemented with keen clinical observation and clinical intuition. With the development of CT and MRI, technologic advances in the computed image-based assessment of adrenocortical carcinoma have been truly remarkable. CT and MRI can help determine whether an adrenal mass is an adrenocortical carcinoma and can also assess for local tumor invasion and metastatic disease. CT and MRI provide the clinician and surgeon with key information to guide medical and surgical management. Three decades from now, what we currently view as conventional imaging (e.g., CT and MRI) will be the imaging equivalents to the plain abdominal roentogram and intravenous pyelogram of the mid-twentieth century.
  

  PMID: 21997291 [PubMed - indexed for MEDLINE]

• 

  The Weiss score and beyond--histopathology for adrenocortical carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  The Weiss score and beyond--histopathology for adrenocortical carcinoma.

  Horm Cancer. 2011 Dec;2(6):333-40

  Authors: Papotti M, Libè R, Duregon E, Volante M, Bertherat J, Tissier F

  Abstract
  The pathological diagnosis of adrenocortical carcinoma (ACC) is still challenging for its rarity and the presence of special variants (pediatric, oncocytic, myxoid, and sarcomatoid). It is based on the recognition at light microscopy of at least three among nine morphological parameters, according to the Weiss scoring system, which has been introduced 27 years ago and nowadays is the most widely employed. Nevertheless, the diagnostic performance of this system is very high but does not reach a sensitivity and specificity of 100%, its diagnostic applicability is potentially low among non-expert pathologists, and a group of borderline cases with only one or two criteria exist of uncertain behavior. Moreover, it is scarcely reproducible in the ACC morphological variants. In fact, specifically for the pure oncocytic neoplasms that seem to have a better prognosis in comparison to the conventional ACCs, a modified system (the Lin-Weiss-Bisceglia) has been proposed. With the aim to simplify the ACC diagnosis, 2 years ago, the "reticulin" diagnostic algorithm has been proposed, based on the observation that the tumoral reticulin framework (highlighted by reticulin silver-based histochemical staining) is consistently disrupted in malignant cases but only in a small subset of benign cases. Following this algorithm, in the presence of reticulin alterations, malignancy is further defined through the identification of at least one of the following parameters: necrosis, high mitotic rate, and venous invasion. As a complement to the morphological approach, some immunohistochemical markers (such as steroidogenic factor 1) have been proposed as diagnostic and prognostic adjuncts but still lack wide clinical validation.
  

  PMID: 21997290 [PubMed - indexed for MEDLINE]

• 

  Unsuspected pheochromocytoma: is it time for a registry?

  Fetched: April 27th, 2012, 11:00pm MDT

  Unsuspected pheochromocytoma: is it time for a registry?

  Can J Anaesth. 2012 Jan;59(1):112-3

  Authors: Mackenzie-Feder J, Tsang J, Demyttenaere S

  PMID: 21989552 [PubMed - indexed for MEDLINE]

• 

  Metronomic therapy concepts in the management of adrenocortical carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  Metronomic therapy concepts in the management of adrenocortical carcinoma.

  Horm Cancer. 2011 Dec;2(6):378-84

  Authors: Berruti A, Sperone P, Bellini E, Daffara F, Perotti P, Ardito A, Saini A, Terzolo M

  Abstract
  Metronomic chemotherapy is the administration of cytotoxic drugs at low doses, on a frequent or continuous schedule, with no extended interruption. This treatment approach can target tumor cells indirectly since it can affect the endothelium of the growing tumor vasculature and stimulates the anticancer immune response. Both the antiangiogenetic and the immunomodulatory roles of metronomic chemotherapy favor a tumor dormancy, a condition that may improve the patient outcome. Prospective clinical trials conducted in several malignancies have shown that metronomic chemotherapy can obtain disease stabilization or responses in tumors that had been made resistant in vivo to conventional chemotherapeutic regimens. Three prospective phase II trials have been conducted in patients with adrenocortical carcinoma (ACC). In all of them, patients heavily pretreated with conventional chemotherapy and mitotane have been enrolled. One trial tested the activity of the association of gemcitabine and fluoropyrimidines administered on a metronomic schedule. In this trial, 40% of patients attained a disease stabilization or disease response that was long lasting in some of them. In the remaining two trials, metronomic chemotherapy was administered in association with antiangiogenetic drugs, and the results were disappointing since no response or stable disease was obtained. In conclusion, metronomic chemotherapy can delay tumor progression in advanced ACC and deserves to be further tested. The concomitant administration of antiangiogenetic drugs may be detrimental. Several important questions remain to be addressed such as the optimal dose and most effective dosing interval, when to use the metronomic approach in the natural history of the disease, the choice of cytotoxic drugs, and the most efficacious way to integrate metronomic chemotherapy with standard therapy protocols.
  

  PMID: 21971765 [PubMed - indexed for MEDLINE]

• 

  When is a phaeo not a phaeo? Depression in an adolescent leading to a phaeochromocytoma-like biochemical profile.

  Fetched: April 27th, 2012, 11:00pm MDT

  When is a phaeo not a phaeo? Depression in an adolescent leading to a phaeochromocytoma-like biochemical profile.

  Clin Endocrinol (Oxf). 2011 Oct;75(4):567-8

  Authors: Yeshayahu Y, Tallett S, Pacak K, De Souza C, Palmert MR

  PMID: 21521331 [PubMed - indexed for MEDLINE]

• 

  Surgical analysis of pediatric and adolescent sporadic pheochromocytoma: single center experience.

  Fetched: April 27th, 2012, 11:00pm MDT

  Surgical analysis of pediatric and adolescent sporadic pheochromocytoma: single center experience.

  Int Urol Nephrol. 2011 Dec;43(4):1019-24

  Authors: Osman Y, Hussein N, Sarhan O, Shorrab AA, Dawaba M, Ghoneim MA

  Abstract
  INTRODUCTION: The aim of this study is to review our experience with sporadic pheochromocytoma in pediatrics and adolescents focusing upon surgical approach, incidence of malignancy, and recurrence rate.
  MATERIALS AND METHODS: Between 1990 and 2007, 8 pediatric patients were diagnosed with sporadic pheochromocytoma. Demographic data, clinical and radiological findings, laboratory profile, preoperative preparation, surgical approach, operative findings, postoperative course as well as pathologic diagnosis of the removed specimen were reviewed.
  RESULTS: Mean age of presentation was 13.1 ± 4.7 years. Five patients had right-sided masses, 1 harbored left-sided mass, and bilaterality was observed in 2 with mean size of 5.7 ± 1.3 cm. Computed tomography showed no evidence of local infiltration, regional lymphadenopathy or distant metastasis in all patients but two. Six masses were excised through thoraco-abdominal approach, 3 were removed laparoscopically, while percutaneous alcohol ablation was adopted for the last. We had one postoperative death (12.5%:1/8), and the remaining 7 patients were followed for a mean of 8.6 ± 3 years. Five patients never had recurrence. Bilateral recurrence developed in 2 patients, where they were safely excised in one patient and was a part of disseminated disease in the other. Malignant nature of the disease was proved in 2 patients and showed poor survival.
  CONCLUSION: Under adequate anesthetic control, pediatric pheochromocytoma could be safely managed through both the open and laparoscopic approaches. Advanced radiological stage would suggest the malignant nature of the disease with dismal outcome. Long-term follow-up is warranted for possibility of delayed curable recurrence.
  

  PMID: 21516474 [PubMed - indexed for MEDLINE]

• 

  Cytochrome P450s in the guinea pig adrenal that are immunologically similar to liver forms: estrogen suppression explains male-female differences.

  Fetched: April 27th, 2012, 11:00pm MDT

  Cytochrome P450s in the guinea pig adrenal that are immunologically similar to liver forms: estrogen suppression explains male-female differences.

  J Steroid Biochem Mol Biol. 1992 Dec;43(8):863-8

  Authors: Black VH

  Abstract
  Several cytochrome P450s have been identified in guinea pig adrenal microsomes which are distinct from the known steroidogenic P450s, c17 and c21, and are immunochemically related to cytochrome P450s found in liver. One, a 52 K protein related to P450 I (CYP1), occurs almost exclusively in males, is localized to the inner zone, and is suppressed by ACTH. Its levels correlate with microsomal capacity for xenobiotic metabolism. The others, related to P450s II and III (CYP2 and 3), are more predominant in males, but not exclusive to them, are found in both the inner and outer zones, and are not suppressed by ACTH. Their functions remain to be elucidated. The male predominance of the CYP1-related protein has recently been shown to be due to suppression of the protein in females by estrogen. To determine if estrogen is also involved in the regulation of the CYP2-related proteins, ovariectomized and sham-operated animals were treated with a long-acting estrogen, estradiol valerate, or with the vehicle alone. These P450s reached male levels in ovariectomized females treated only with the vehicle. Their enhanced levels were suppressed by treatment with estrogen. Estrogen treatment also suppressed the levels of the P450s seen in sham-operated females. Endogenous estrogen produced similar effects. In hemi-ovariectomized females the contralateral ovary hypertrophied, a state in which estrogen levels would be maintained or increased. In these females no increase occurred in the immunodetectable P450s. In normal females, estrogen levels are low in prepubertal animals, rise at the time of puberty and drop again after ovarian cycling is completed. The CYP2-related proteins were present in adrenal microsomes of prepubertal females, but were suppressed after puberty. On the other hand, post-estrous females, in whom estrogen levels would be low, acquired male levels of these proteins in their adrenal microsomes. P450c17 and P450c21, as well as 3β-hydroxysteroid dehydrogenase, were not affected by surgery or estrogen. Taken together, these experiments indicate that suppression by estrogen in females can account, in large part, for the predominance of several immunochemical homologs of liver P450s in adult male guinea pig adrenals.
  

  PMID: 22217830 [PubMed - indexed for MEDLINE]

• 

  Neuroblastoma stage 4S: a multifocal stem-cell disease of the developing neural crest.

  Fetched: April 27th, 2012, 11:00pm MDT

  Neuroblastoma stage 4S: a multifocal stem-cell disease of the developing neural crest.

  Lancet Oncol. 2012 Mar;13(3):229-30

  Authors: van Noesel MM

  PMID: 22381931 [PubMed - indexed for MEDLINE]

• 

  Hirsutism in women.

  Fetched: April 27th, 2012, 11:00pm MDT

  Hirsutism in women.

  Am Fam Physician. 2012 Feb 15;85(4):373-80

  Authors: Bode D, Seehusen DA, Baird D

  Abstract
  Hirsutism is excess terminal hair that commonly appears in a male pattern in women. Although hirsutism is generally associated with hyperandrogenemia, one-half of women with mild symptoms have normal androgen levels. The most common cause of hirsutism is polycystic ovary syndrome, accounting for three out of every four cases. Many medications can also cause hirsutism. In patients whose hirsutism is not related to medication use, evaluation is focused on testing for endocrinopathies and neoplasms, such as polycystic ovary syndrome, adrenal hyperplasia, thyroid dysfunction, Cushing syndrome, and androgen-secreting tumors. Symptoms and findings suggestive of neoplasm include rapid onset of symptoms, signs of virilization, and a palpable abdominal or pelvic mass. Patients without these findings who have mild symptoms and normal menses can be treated empirically. For patients with moderate or severe symptoms, an early morning total testosterone level should be obtained, and if moderately elevated, it should be followed by a plasma free testosterone level. A total testosterone level greater than 200 ng per dL (6.94 nmol per L) should prompt evaluation for an androgen-secreting tumor. Further workup is guided by history and physical examination, and may include thyroid function tests, prolactin level, 17-hydroxyprogesterone level, and corticotropin stimulation test. Treatment includes hair removal and pharmacologic measures. Shaving is effective but needs to be repeated often. Evidence for the effectiveness of electrolysis and laser therapy is limited. In patients who are not planning a pregnancy, first-line pharmacologic treatment should include oral contraceptives. Topical agents, such as eflornithine, may also be used. Treatment response should be monitored for at least six months before making adjustments.
  

  PMID: 22335316 [PubMed - indexed for MEDLINE]

• 

  Is there an optimal scan time for 6-[F-18]fluoro-L-DOPA PET in pheochromocytomas and paragangliomas?

  Fetched: April 27th, 2012, 11:00pm MDT

  Is there an optimal scan time for 6-[F-18]fluoro-L-DOPA PET in pheochromocytomas and paragangliomas?

  Clin Nucl Med. 2012 Feb;37(2):e24-9

  Authors: Hentschel M, Rottenburger C, Boedeker CC, Neumann HP, Brink I

  Abstract
  PURPOSE: To define the appropriate scan time for fluorine-18-labeled dihydroxyphenylalanine (F-18 DOPA) PET in oncological imaging of pheochromocytomas and paragangliomas.
  MATERIALS AND METHODS: F-18 DOPA PET examinations were performed in 9 patients with 7 pheochromocytomas and 4 head and neck paragangliomas using a dedicated PET scanner. The acquisition started with a dynamic single-bed scan in the tumor region over the first 60 minutes after tracer injection followed by a late time whole-body scan at approximately 130 minutes. Standard uptake values (SUVs) were calculated in tumors, surrounding background, and adjacent normal tissues of relevance. Furthermore, kinetic analysis was performed using a 2-compartment model with rate constants for uptake (K1'), release (k2'), metabolism (k3'), and reverse reaction (k4') for region of interest and pixel-wise analysis.
  RESULTS: All tumors show a marked increased F-18 DOPA uptake, which was visually detectable and distinguishable from the surrounding tissue. The SUV is significantly lower in neck paraganglioma compared with abdominal pheochromocytomas. Mean time-activity curves of F-18 DOPA in tumors show a rapid uptake of the tracer. Already 2 minutes after the injection, the activity in the tumor is beyond that of the blood pool. The average maximum value (SUVmean = 8.2) has already been reached after 20 minutes. Afterward, a very slight decrease of the tumor SUV starts, which still amounts to 80% of the maximum value after 132 minutes. Due to the continuous decrease of activity in the background tissue, the tumor-to-background ratio of SUVs shows a constant increase within the entire period of examination. The mean values of apparent kinetic constants obtained by region of interest analysis averaged over all tumors are as follows: K1' = 2.89 ± 2.56 min(-1), k2' = 2.59 ± 2.81 min(-1), k3' = 0.301 ± 0.395 min(-1), and k4' = 0.044 ± 0.043 min(-1).
  CONCLUSIONS: Pheochromocytoma and paraganglioma take up F-18 DOPA very quickly. At best, the acquisition for static clinical PET imaging of paraganglioma with F-18 DOPA can start at 20 minutes postinjection for maximum uptake in tumors. Separation of tumor, background, and adjacent normal tissues is feasible due to their differences in SUV values and kinetics. The kinetic analysis demonstrates an F-18 DOPA accumulation within the tumor due to considerable differences between the rate constants of uptake and metabolism. Second, in contradiction to healthy brain, paraganglionic tumors show a reversible F-18 DOPA metabolism.
  

  PMID: 22228360 [PubMed - indexed for MEDLINE]

• 

  Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma.

  Eur J Endocrinol. 2012 Mar;166(3):451-8

  Authors: Berruti A, Sperone P, Ferrero A, Germano A, Ardito A, Priola AM, De Francia S, Volante M, Daffara F, Generali D, Leboulleux S, Perotti P, Baudin E, Papotti M, Terzolo M

  Abstract
  BACKGROUND: There is a strong rationale in the use of antiangiogenic therapy in the management of adrenocortical carcinoma (ACC). Metronomic administration of chemotherapy and antiangiogenic drugs can be synergistic in targeting endothelial cells.
  OBJECTIVE: We assessed the activity of sorafenib plus metronomic paclitaxel as second/third-line therapy in advanced ACC patients. We also tested the activity of sorafenib and paclitaxel against NCI-H295R in vitro.
  DESIGN: Multicenter, prospective phase II trial. Setting Referral centers for ACC.
  METHODS: Twenty-five consecutive metastatic ACC patients who progressed after mitotane plus one or two chemotherapy lines were planned to be enrolled. The patients received a combination of i.v. paclitaxel (60 mg/m(2) every week) and oral sorafenib (400 mg twice a day) till progression. The primary aim was to measure the progression-free survival rate after 4 months and the secondary aims were to assess the objective response rate and toxicity.
  RESULTS: Tumor progression was observed in nine evaluable patients at the first assessment. These results led to the premature interruption of the trial. The treatment was well tolerated. The most relevant toxicities were fatigue, being grade 2 or 3 in four patients, and hypophosphatemia, being grade 3 in three patients. In the in vitro study, sorafenib impaired the viability of H295R cells with dose-response and time-response relationships. The in vitro sorafenib activity was not increased in combination with paclitaxel.
  CONCLUSIONS: Despite the in vitro activity, sorafenib plus weekly paclitaxel is an inactive salvage treatment in patients with advanced ACC and should not be recommended.
  

  PMID: 22189997 [PubMed - indexed for MEDLINE]

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  Targeted therapies for adrenocortical carcinoma: IGF and beyond.

  Fetched: April 27th, 2012, 11:00pm MDT

  Targeted therapies for adrenocortical carcinoma: IGF and beyond.

  Horm Cancer. 2011 Dec;2(6):385-92

  Authors: Demeure MJ, Bussey KJ, Kirschner LS

  Abstract
  Standard chemotherapy for adrenocortical cancer currently is under evaluation in the context of the recently completed FIRM-ACT evaluating the combination of mitotane with either streptozocin or etoposide, cisplatin, and doxorubicin. New agents are eagerly sought by the ACC community that hopes to make progress against this deadly disease. Investigators have begun to dissect the molecular and genomic context of ACC with a goal of identifying potential novel therapeutic agents. One gene consistently overexpressed in ACC is insulin growth factor type 2. Targeting its receptor IGF1R has shown encouraging results in ACC cell lines and against murine xenografts. As a result, clinical trials to evaluate agents targeting the IGF1R have been done including mitotane and IMC-A12 (a monoclonal antibody) and the GALACCTIC trial that has just completed accrual to evaluate OSI-906, a small molecule IGF1R antagonist. On the horizon are other agents targeting other tyrosine kinases, including EGF and FGF, and novel strategies such as individualized tumor analysis to select treatment.
  

  PMID: 22170383 [PubMed - indexed for MEDLINE]

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  Syndrome of inappropriate secretion of antidiuretic hormone caused by pituitary macroadenoma with hemangiomatous stroma.

  Fetched: April 27th, 2012, 11:00pm MDT

  Syndrome of inappropriate secretion of antidiuretic hormone caused by pituitary macroadenoma with hemangiomatous stroma.

  Tokai J Exp Clin Med. 2011 Dec;36(4):128-33

  Authors: Sato H, Takahashi H, Kanai G, Kakuta T, Itoh J, Inomoto C, Osamura RY

  Abstract
  A 55-year-old Japanese man was referred to our hospital because of disturbance of consciousness and hyponatremia. He had been aware of general fatigue, nausea, and headache for two weeks. Tests revealed hyponatremia, plasma hypoosmolarity with urine hyperosmolarity, an elevated level of urine sodium excretion, and normal functions of the kidney, adrenal gland, and thyroid. These findings were compatible with syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Head magnetic resonance imaging (MRI) demonstrated a pituitary tumor measuring 20 x 22 x 21 mm that pushed the pituitary stalk upward. Endocrinological evaluations suggested that the pituitary adenoma was non-functional. The pituitary adenoma was surgically removed, and histological examination revealed a biphasic appearance characterized by endocrine cells and a hemangiomatous stroma. After surgery, the patient developed pituitary hypothyroidism, pituitary adrenal insufficiency, and pituitary gonadal failure. Therefore, levothyroxine sodium, 50 µg per day, and hydrocortisone, 10 mg per day, were administered orally. Androgen depot, 250 mg every two months, was also injected intramuscularly. The hyponatremia did not recur, and the patient has done well for the last five years. The pituitary adenoma in this case showed two features: one was the cause of SIADH, and the other was a biphasic histological picture of endocrine cells with a hemangiomatous stroma.
  

  PMID: 22167496 [PubMed - indexed for MEDLINE]

• 

  Staging and Functional Characterization of Pheochromocytoma and Paraganglioma by 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography.

  Fetched: April 27th, 2012, 11:00pm MDT

  Staging and Functional Characterization of Pheochromocytoma and Paraganglioma by 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography.

  J Natl Cancer Inst. 2012 Apr 18;

  Authors: Timmers HJ, Chen CC, Carrasquillo JA, Whatley M, Ling A, Eisenhofer G, King KS, Rao JU, Wesley RA, Adams KT, Pacak K

  Abstract
  BackgroundPheochromocytomas and paragangliomas (PPGLs) are rare tumors of the adrenal medulla and extra-adrenal sympathetic chromaffin tissues; their anatomical and functional imaging are critical to guiding treatment decisions. This study aimed to compare the sensitivity and specificity of (18)F-fluorodeoxyglucose positron emission tomography with computed tomography ((18)F-FDG PET/CT) for tumor localization and staging of PPGLs with that of conventional imaging by [(123)I]-metaiodobenzylguanidine single photon emission CT ((123)I-MIBG SPECT), CT, and magnetic resonance imaging (MRI).MethodsA total of 216 patients (106 men, 110 women, aged 45.2 ± 14.9 years) with suspected PPGL underwent CT or MRI, (18)F-FDG PET/CT, and (123)I-MIBG SPECT/CT. Sensitivity and specificity were measured as endpoints and compared by the McNemar test, using two-sided P values only.ResultsSixty (28%) of patients had nonmetastatic PPGL, 95 (44%) had metastatic PPGL, and 61 (28%) were PPGL negative. For nonmetastatic tumors, the sensitivity of (18)F-FDG was similar to that of (123)I-MIBG but less than that of CT/MRI (sensitivity of (18)F-FDG = 76.8%; of (123)I-MIBG = 75.0%; of CT/MRI = 95.7%; (18)F-FDG vs (123)I-MIBG: difference = 1.8%, 95% confidence interval [CI] = -14.8% to 14.8%, P = .210; (18)F-FDG vs CT/MRI: difference = 18.9%, 95% CI = 9.4% to 28.3%, P < .001). The specificity was 90.2% for (18)F-FDG, 91.8% for (123)I-MIBG, and 90.2% for CT/MRI. (18)F-FDG uptake was higher in succinate dehydrogenase complex- and von Hippel-Lindau syndrome-related tumors than in multiple endocrine neoplasia type 2 (MEN2) related tumors. For metastases, sensitivity was greater for (18)F-FDG and CT/MRI than for (123)I-MIBG (sensitivity of (18)F-FDG = 82.5%; of (123)I-MIBG = 50.0%; of CT/MRI = 74.4%; (18)F-FDG vs (123)I-MIBG: difference = 32.5%, 95% CI = 22.3% to 42.5%, P < .001; CT/MRI vs (123)I-MIBG: difference = 24.4%, 95% CI = 11.3% to 31.6%, P < .001). For bone metastases, (18)F-FDG was more sensitive than CT/MRI (sensitivity of (18)F-FDG = 93.7%; of CT/MRI = 76.7%; difference = 17.0%, 95% CI = 4.9% to 28.5%, P = .013).ConclusionsCompared with (123)I-MIBG SPECT and CT/MRI, both considered gold standards for PPGL imaging, metastases were better detected by (18)F-FDG PET. (18)F-FDG PET provides a high specificity in patients with a biochemically established diagnosis of PPGL.
  

  PMID: 22517990 [PubMed - as supplied by publisher]

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  [The Conceptual Oligometastatic Non-small Cell Lung Cancer and Therapeutic Strategies].

  Fetched: April 20th, 2012, 11:00pm MDT

  [The Conceptual Oligometastatic Non-small Cell Lung Cancer and Therapeutic Strategies].

  Zhongguo Fei Ai Za Zhi. 2012 Apr 20;15(4):242-5

  Authors: Kang X, Chen K

  Abstract
  Non-small cell lung cancer (NSCLC) ranks among the most prevalent malignancies and is the major cause of cancer-related deaths worldwide. Nearly 20%-50% will accompany by metastatic disease and the most common extrapulmonary sites of distant metastases are the brain, bone, liver and adrenal gland. The oligometastatic state is a biologically mild tumor stage and a intermediate state in which spread may be limited to specific organs and metastases might be present in limited numbers. Oligometastases are thought to arise from micrometastases, which have been dormant for a period of time. Local control may be an crucial component of a curative therapeutic strategy in the following four clinical schemes: to prohibit metastases; to cure occult metastatic disease; to remedy oligometastases; and to deracinate any residual lesion after systemic therapy. This review aims to outline the concept of the oligometastatic NSCLC and its strategies of treatment.
  

  PMID: 22510511 [PubMed - in process]

• 

  Role of K(+) channels in the pathophysiology of primary aldosteronism.

  Fetched: April 19th, 2012, 11:00pm MDT

  Role of K(+) channels in the pathophysiology of primary aldosteronism.

  Hypertension. 2012 Mar;59(3):534-6

  Authors: Carey RM

  PMID: 22252399 [PubMed - indexed for MEDLINE]

Trial - Adrenocortical Carcinoma

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  Seneca Valley Virus-001 and Cyclophosphamide in Treating Young Patients With Relapsed or Refractory Neuroblastoma, Rhabdomyosarcoma, or Rare Tumors With Neuroendocrine Features

  Posted: January 13th, 2010, 8:00am MST

  Conditions:   Adrenocortical Carcinoma;   Gastrointestinal Carcinoid Tumor;   Kidney Cancer;   Neuroblastoma;   Retinoblastoma;   Sarcoma
  Interventions:   Biological: Seneca Valley virus-001;   Drug: cyclophosphamide;   Other: laboratory biomarker analysis;   Other: pharmacological study
  Sponsors:   National Cancer Institute (NCI);   Children's Oncology Group;   National Cancer Institute (NCI)
  Recruiting - verified May 2012

Google - Adrenocortical Carcinoma

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  Adrenalectomy Plus Nephrectomy Linked to Reduced Survival - Medscape

  Posted: May 19th, 2012, 9:04am MDT

  Adrenalectomy Plus Nephrectomy Linked to Reduced Survival Medscape May 19, 2012 (Atlanta, Georgia) — Overall survival is lower in patients with renal cell carcinoma who underwent radical nephrectomy with concurrent ipsilateral adrenalectomy than in patients with the ipsilateral adrenal gland left intact, according to ... and more »

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  Large-scale study finds treatment standard for rare cancer - HealthCanal.com

  Posted: May 16th, 2012, 2:07pm MDT

  Large-scale study finds treatment standard for rare cancer HealthCanal.com Initial results from a first-of-its-kind international consortium of adrenal cancer researchers suggest there might be a treatment standard for patients with this extremely rare and deadly disease. The consortium – which grew out of an international ... and more »

• 

  Large-scale Study Finds Treatment Standard for Rare Cancer - HealthNewsDigest.com

  Posted: May 16th, 2012, 7:32am MDT

  HealthNewsDigest.com

  Large-scale Study Finds Treatment Standard for Rare Cancer HealthNewsDigest.com By Staff Editor (HealthNewsDigest.com) - Initial results from a first-of-its-kind international consortium of adrenal cancer researchers suggest there might be a treatment standard for patients with this extremely rare and deadly disease. and more »

• 

  Large-scale study finds treatment standard for rare cancer - University of Michigan Health System News (press release)

  Posted: May 16th, 2012, 5:47am MDT

  University of Michigan Health System News (press release)

  Large-scale study finds treatment standard for rare cancer University of Michigan Health System News (press release) Initial results from a first-of-its-kind international consortium of adrenal cancer researchers suggest there might be a treatment standard for patients with this extremely rare and deadly disease. The consortium – which grew out of an international ... and more »

• 

  Fighting for Coach Moose - Cherry Hill Courier Post

  Posted: May 14th, 2012, 11:15pm MDT

  Fighting for Coach Moose Cherry Hill Courier Post Last month, Musumeci learned the cancer had spread. According to the National Cancer Institute, an adrenocortical carcinoma is a rare tumor that affects only up to 2 persons per one million and is most common in children younger than 5 and adults in ...

• 

  Marathon to battle cancer - Dandenong Star

  Posted: May 10th, 2012, 3:05pm MDT

  Marathon to battle cancer Dandenong Star The Dandenong woman has thrown her support behind good friend and former co-worker Gervais Serret, who is battling adrenocortical carcinoma, an extremely rare and aggressive cancer. Last year, Katarina dedicated time and effort into writing a ...

• 

  Combination chemotherapy shows benefits for adrenal cancer patients - Medical Xpress

  Posted: May 4th, 2012, 12:29am MDT

  Combination chemotherapy shows benefits for adrenal cancer patients Medical Xpress Endocrinologists at the University of Birmingham have played a key role in a major international clinical trial which has found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up ... and more »

• 

  Combination chemotherapy shows benefits for adrenal cancer patients - HealthCanal.com

  Posted: May 3rd, 2012, 1:25pm MDT

  Combination chemotherapy shows benefits for adrenal cancer patients HealthCanal.com Endocrinologists at the University of Birmingham have played a key role in a major international clinical trial which has found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up ... and more »

• 

  Research and Markets: Adrenocortical Carcinoma (Adrenal Cortex Cancer ... - SunHerald.com

  Posted: May 3rd, 2012, 12:40pm MDT

  Research and Markets: Adrenocortical Carcinoma (Adrenal Cortex Cancer ... SunHerald.com DUBLIN -- Research and Markets [www.researchandmarkets.com] has announced the addition of Global Markets Direct's new report "Adrenocortical Carcinoma (Adrenal Cortex Cancer) - Pipeline Review, ... and more »

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  Mitotane plus Chemo Combo May Benefit Adrenocortical Cancer - MD News (press release)

  Posted: May 3rd, 2012, 6:33am MDT

  Mitotane plus Chemo Combo May Benefit Adrenocortical Cancer MD News (press release) For patients with advanced adrenocortical cancer, mitotane plus a combination of etoposide, doxorubicin, and cisplatin is superior to streptozocin-mitotane for rates of response and progression-free survival, but does not improve overall survival. and more »

• 

  Mitotane Plus Chemo Combo May Benefit Patients with Adrenocortical Cancer - Renal and Urology News

  Posted: May 3rd, 2012, 6:26am MDT

  Mitotane Plus Chemo Combo May Benefit Patients with Adrenocortical Cancer Renal and Urology News For patients with advanced adrenocortical cancer, mitotane plus a combination of etoposide, doxorubicin, and cisplatin (EDP) is superior to streptozocin-mitotane for rates of response and progression-free survival, but does not improve overall survival ... and more »

• 

  Combination chemo holds back cancer - Public Service

  Posted: May 3rd, 2012, 2:57am MDT

  Combination chemo holds back cancer Public Service Endocrinologists at the University of Birmingham have found that giving a combination of chemotherapy drugs to patients suffering from advanced adrenal cancer can help them to live up to a fifth longer. According to the New England Journal of Medicine, ... and more »

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  Mixed Results for New Tx in Adrenal Cancer - MedPage Today

  Posted: May 2nd, 2012, 4:05pm MDT

  Mixed Results for New Tx in Adrenal Cancer MedPage Today Patients with advanced adrenocortical carcinoma lived no longer when standard therapy was combined with three-agent chemotherapy but had a significantly higher response rate and progression-free survival (PFS). Note that the three-drug combination did ... and more »

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  Mitotane + Chemo Combo May Benefit Adrenocortical Cancer - Doctors Lounge

  Posted: May 2nd, 2012, 4:03pm MDT

  Mitotane + Chemo Combo May Benefit Adrenocortical Cancer Doctors Lounge For patients with advanced adrenocortical cancer, mitotane plus a combination of etoposide, doxorubicin, and cisplatin is superior to streptozocin-mitotane for rates of response and progression-free survival, but does not improve overall survival, ... and more »

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  Holland family looks for second opinion in cancer fight - HollandSentinel.com

  Posted: April 7th, 2012, 11:23am MDT

  Holland family looks for second opinion in cancer fight HollandSentinel.com She said doctors at Helen DeVos Children's Hospital have said his adrenal carcinoma has returned and not much more can be done. Here Jesus is pictured during one hospital stay, when a pet therapy dog visited. By PEG MCNICHOL For two weekends, ...

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  The R337H mutation in TP53 and breast cancer in Brazil - 7thSpace Interactive (press release)

  Posted: March 28th, 2012, 4:44am MDT

  The R337H mutation in TP53 and breast cancer in Brazil 7thSpace Interactive (press release) Germline mutations in p53 are associated with the Li-Fraumeni Syndrome which is characterized by childhood cancers, including pediatric adrenal cortical carcinomas and early onset breast cancer. The high incidence of adrenal cortical carcinomas in ...

PubMed - Adrenocortical Carcinoma

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  Adrenal gland: Adrenocortical carcinoma-first RCT.

  Fetched: May 24th, 2012, 11:00pm MDT

  Adrenal gland: Adrenocortical carcinoma-first RCT.

  Nat Rev Endocrinol. 2012 May 22;

  Authors: Barranco C

  PMID: 22614717 [PubMed - as supplied by publisher]

• 

  KCNQ1OT1 hypomethylation: A Novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?

  Fetched: May 24th, 2012, 11:00pm MDT

  KCNQ1OT1 hypomethylation: A Novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?

  Pediatr Blood Cancer. 2011 Dec 11;

  Authors: Wijnen M, Alders M, Zwaan CM, Wagner A, Cheng FW, van den Heuvel-Eibrink MM

  Abstract
  Pediatric adrenal tumors, other than neuroblastoma, are rare and can be associated with a genetic predisposition. In this report we describe two patients with an isolated and apparently sporadic adrenocortical tumor; one girl with a carcinoma, the other girl with an adenoma. In both patients genetic screening revealed hypomethylation of the KCNQ1OT1 gene, well-known for its association with the Beckwith-Wiedemann syndrome. This represents a likely novel genetic predisposition in patients with adrenocortical tumors without clear phenotypic features of the Beckwith-Wiedemann syndrome. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc.
  

  PMID: 22610651 [PubMed - as supplied by publisher]

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  Positional statement of the European Society of Endocrine Surgeons (ESES) on malignant adrenal tumors.

  Fetched: May 24th, 2012, 11:00pm MDT

  Positional statement of the European Society of Endocrine Surgeons (ESES) on malignant adrenal tumors.

  Langenbecks Arch Surg. 2012 Feb;397(2):145-6

  Authors: Henry JF, Peix JL, Kraimps JL

  PMID: 22203016 [PubMed - indexed for MEDLINE]

• 

  Clinical impact of TP53 alterations in adrenocortical carcinomas.

  Fetched: May 24th, 2012, 11:00pm MDT

  Clinical impact of TP53 alterations in adrenocortical carcinomas.

  Langenbecks Arch Surg. 2012 Feb;397(2):209-16

  Authors: Waldmann J, Patsalis N, Fendrich V, Langer P, Saeger W, Chaloupka B, Ramaswamy A, Fassnacht M, Bartsch DK, Slater EP

  Abstract
  BACKGROUND: To evaluate the role of somatic TP53 mutations and to correlate somatic and germline mutations with results of immunostaining, a large cohort of ACC patients was analyzed.
  PATIENTS AND METHODS: Patients with ACC who underwent potential curative surgery at the authors' department were screened for TP53 somatic and germline mutations in exons 5, 6, 7, 8, and 10 by DHPLC analysis. Aberrant samples were further analyzed by direct sequencing. Immunostaining was performed on corresponding paraffin sections in all patients. Complete clinical and follow-up data were correlated with the status of TP53.
  RESULTS: Thirty ACC patients were included. Four of 30 patients showed aberrant DHPLC configuration and direct sequencing confirmed 2 (7%) germline mutations (R337H, R248W), 1 (3%) somatic mutation (R213X), and 1 (3%) noncoding polymorphism (g.17708 A>T). The only patient with a positive family history harbored a TP53 mutation. Tumors of the three patients with mutations showed aberrant p53 expression in more than 10% of cells by immunostaining, compared to only 3 of 27 patients without mutations (p = 0.009). Aberrant p53 expression (>5%) was detected in 12/30 (40%) ACCs. The latter was associated with an increased Ki67 and van Slooten index (p ≤ 0.001; p = 0.020). Disease-free survival decreased significantly in patients with aberrant p53 IHC of more than 5% of cells (65.7 ± 12.4 vs. 26.6 ± 8.7 months; p = 0.043 log rank test).
  CONCLUSIONS: Patients with ACC revealed aberrant expression of p53 in 40%, and mutations were identified in 25% of these patients. Therefore aberrant p53 expression should be considered an indicator for genetic testing. A subgroup of apparently sporadic ACC is caused by TP53 germline mutations, and family history is a strong indicator for p53 germline mutations.
  

  PMID: 22203015 [PubMed - indexed for MEDLINE]

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  Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava--a European Society of Endocrine Surgeons (ESES) survey.

  Fetched: May 24th, 2012, 11:00pm MDT

  Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava--a European Society of Endocrine Surgeons (ESES) survey.

  Langenbecks Arch Surg. 2012 Feb;397(2):225-31

  Authors: Mihai R, Iacobone M, Makay O, Moreno P, Frilling A, Kraimps JL, Soriano A, Villar del Moral J, Barczynski M, Durán MC, Sadler GP, Niederle B, Dralle H, Harrison B, Carnaille B

  Abstract
  BACKGROUND: Most patients with adrenocortical cancer (ACC) continue to present with advanced disease. Invasion into the inferior vena cava (IVC) defines stage III disease and the management of such patients raises additional difficulties.
  METHOD: A multicentre survey was organized by emailing a standardized proforma to members of the European Society of Endocrine Surgery (ESES). Anonymised retrospective clinical data were collected.
  RESULTS: Replies were received from 18 centres in nine countries. ACC with IVC invasion was encountered in 38 patients (18F:20M, age 15-84 years, median 54 years). There were 16 nonfunctioning tumours and 22 functioning tumours predominantly right-sided (26R:12L) and measuring 18-255 mm (median 115 mm). Fourteen patients had metastatic disease at presentation. Tumour thrombus extended in the prehepatic IVC (n = 21), subdiaphragmatic IVC (n = 6) or into the SVC/right atrium (n = 3). Open adrenalectomy was associated with resection of surrounding viscera in 24 patients (nephrectomy n = 16, liver resection n = 14, splenectomy n = 3, Whipple procedure n = 2). IVC was controlled locally (n = 27), at suprahepatic levels (n = 6) or necessitated cardiac bypass (n = 5). Complete resection (R0, n = 20) was achieved in the majority of patients, with a minority having microscopic persistent disease (R1, n = 7) or macroscopic residual disease (R2, n = 4). Perioperative 30-day mortality was 13% (n = 5). Postoperative Mitotane was used in 23 patients and chemotherapy in eight patients. Twenty-five patients died 2-61 months after their operation (median 5 months). Currently, 13 patients are alive at 2-58 months (median 16 months) with known metastatic disease (n = 7) or with no signs of distant disease (n = 6).
  CONCLUSION: This dataset is limited by the lack of a denominator as it remains unknown how many other patients with ACC presenting with IVC invasion did not undergo surgery. The relatively low perioperative mortality and the long disease-free survival achieved by some patients should encourage surgeons with adequate experience to offer surgical treatment to patients presenting with advanced adrenocortical cancers.
  

  PMID: 22134748 [PubMed - indexed for MEDLINE]

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  [German adrenocortical carcinoma registry : Surgical therapy results and follow-up treatment.]

  Fetched: May 17th, 2012, 11:00pm MDT

  [German adrenocortical carcinoma registry : Surgical therapy results and follow-up treatment.]

  Chirurg. 2012 May 16;

  Authors: Reibetanz J, Kroiss M, Deutschbein T, Fenske W, Gasser M, Jurowich C, Germer CT, Allolio B, Fassnacht M

  Abstract
  Adrenocortical carcinoma (ACC) is a highly aggressive endocrine disease with an incidence of 1-2 cases per million population per year. Due to the low incidence of ACC knowledge concerning the surgical management is mainly based on retrospective studies or recommendations of isolated experts. Cancer databases, such as the German ACC registry are prerequisite to collect and evaluate clinical data from a large number of patients. For non-metastatic tumor stages, complete tumor resection is the only treatment with curative intent. Open surgery remains the recommended approach for ACC. However, in small tumors with uncertain malignancy a laparoscopic resection by an expert surgeon can be considered. A loco-regional lymphadenectomy should be part of the primary surgical treatment of ACC. Tumor recurrence is common even after an apparently complete primary resection. Therefore, based on the individual risk (tumor size, resection status, proliferation index) adjuvant mitotane treatment is recommended in most patients. Patients with low-risk should be included in the ADIUVO trial. In case of tumor relapse indications for a reoperation should be strongly considered, especially when the time interval since the primary surgery is long (> 12 months) and a complete resection of the recurrent disease seems to be feasible.
  

  PMID: 22585346 [PubMed - as supplied by publisher]

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  Neuroendocrine carcinoma in an adolescent with hypercortisolemia.

  Fetched: May 17th, 2012, 11:00pm MDT

  Neuroendocrine carcinoma in an adolescent with hypercortisolemia.

  J Pediatr Hematol Oncol. 2012 Apr;34(3):e117-9

  Authors: Fagan EL, Slone JS, Shoemaker AH, Black J, Berlin J, E Engel M

  Abstract
  We present a 16-year-old boy with weakness, hypercortisolemia, and markedly elevated adrenocorticotropic hormone. Computed tomographic imaging revealed hepatic lesions and a calcified pancreatic mass. Biopsy of the hepatic lesions revealed moderately differentiated neuroendocrine carcinoma. The primary tumor could not be determined. The patient received neoadjuvant chemotherapy with carboplatin and etoposide followed by therapeutic bilateral adrenalectomy and tumor debulking. Despite significant clinical improvement, restaging revealed progressive hepatic disease. The patient died 9 months after diagnosis. Autopsy revealed disseminated neuroendocrine carcinoma. The rarity of this tumor compels a cooperative investigational model involving pediatric and adult oncologists.
  

  PMID: 22441712 [PubMed - indexed for MEDLINE]

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  Differentiation between benign and malignant adrenal mass using contrast-enhanced ultrasound.

  Fetched: May 16th, 2012, 11:00pm MDT

  Differentiation between benign and malignant adrenal mass using contrast-enhanced ultrasound.

  Ultraschall Med. 2011 Oct;32(5):460-71

  Authors: Friedrich-Rust M, Glasemann T, Polta A, Eichler K, Holzer K, Kriener S, Herrmann E, Nierhoff J, Bon D, Bechstein WO, Vogl T, Zeuzem S, Bojunga J

  Abstract
  PURPOSE: Adrenal masses can be detected by ultrasound with high sensitivity and specificity. The aim of the present study was to evaluate CEUS in a large patient population using CEUS patterns identified in a previous pilot study.
  MATERIALS AND METHODS: 116 adrenal masses were evaluated by ultrasound, including CEUS with the contrast agent Sonovue®. The dynamic of contrast enhancement (CE) was analyzed using time-intensity curves. The time of the first CE in the adrenal mass was used to define four CEUS patterns: pattern I = early arterial CE, pattern II = arterial CE, pattern III = late CE, pattern IV = no CE. In addition, all patients received CT/MRI and hormonal testing. In suspicious cases biopsy or adrenalectomy was performed.
  RESULTS: CEUS patterns I&II were seen in all patients with primary or secondary malignant lesions of the adrenal gland (n = 16). The sensitivity and specificity of CEUS for the diagnosis of malignant adrenal mass were 100 % (CI [75;100]) and 67 % (CI [56;75]), respectively. Overall histology was available as a reference method for 40 adrenal masses. In 68 % of histologically diagnosed adrenal masses, MRI/CT and CEUS were congruent concerning the characterization of malignant versus benign adrenal mass.
  CONCLUSION: Contrast-enhanced ultrasound may be a useful method in the diagnostic work-up of adrenal mass with excellent sensitivity for the diagnosis of malignancy.
  

  PMID: 21667434 [PubMed - indexed for MEDLINE]

• 

  Interleukin-13 receptor alpha2 is a novel therapeutic target for human adrenocortical carcinoma.

  Fetched: May 11th, 2012, 11:00pm MDT

  Interleukin-13 receptor alpha2 is a novel therapeutic target for human adrenocortical carcinoma.

  Cancer. 2012 May 8;

  Authors: Jain M, Zhang L, He M, Patterson EE, Nilubol N, Fojo AT, Joshi B, Puri R, Kebebew E

  Abstract
  BACKGROUND.: Adrenocortical carcinoma (ACC) is a relatively rare but aggressive malignancy with limited therapeutic options. Previous genome-wide expression studies have demonstrated the overexpression of interleukin-13 receptor alpha2 (IL13Rα2) in some human malignancies. METHODS.: The authors evaluated IL13Rα2 mRNA and protein expression in 21 normal samples, 78 benign samples, 10 primary malignant samples, and 25 metastatic/recurrent samples and performed functional analyses with IL13 ligand and IL13 Rα2 knockdown in vitro. The sensitivity of 2 ACC cell lines (NCI-H295R [high IL13Rα2 expression] and SW13 [low IL13Rα2 expression]) to a highly specific IL-13 conjugated with Pseudomonas exotoxin (IL-13-PE) also was evaluated in both in vitro and in vivo models. RESULTS.: IL13Rα2 was overexpressed in malignant tumors compared with benign and normal samples (15-fold higher; P < .05). Immunohistochemistry also confirmed higher protein expression in malignant and benign tumors than in normal adrenocortical tissues (P < .05). The half-maximal inhibitory concentration for IL-13-PE was 1.3 ng/mL in the NCI-H295R cell line and 1000 ng/mL in the SW13 cell line. Mice that received intratumoral or intraperitoneal IL-13-PE injection had a significant reduction in tumor size and increased tumor necrosis compared with control groups (P < .05) and also had prolonged survival (P < .05). IL13Rα2 protein expression increased in cells that were treated with IL-13 ligand along with cell invasion (P < .05). Direct IL13Rα2 knockdown decreased cellular proliferation and invasion (P < .05). CONCLUSIONS.: The current results indicated that IL13Rα2 is overexpressed in ACC and regulates cell invasion and proliferation. IL13Rα2 is a novel therapeutic target for the treatment of human ACC. Cancer 2012. © 2012 American Cancer Society.
  

  PMID: 22570059 [PubMed - as supplied by publisher]

• 

  The molecular basis of adrenocortical cancer.

  Fetched: May 10th, 2012, 11:00pm MDT

  The molecular basis of adrenocortical cancer.

  Cancer Genet. 2012 Apr;205(4):131-7

  Authors: Lehmann T, Wrzesinski T

  Abstract
  Adrenocortical tumors (ACTs) are common, and most are benign adrenocortical adenomas (ACAs). Malignant adrenocortical carcinoma (ACC) is a rare tumor type and is observed at the rate of one or two cases per million annually. ACTs are classified as either ACAs or ACCs by histopathologic methods that are based on nine Weiss scoring criteria, including the nuclear grade, mitotic rate, presence of necrosis, and others. In this review, we describe the findings of studies that have examined the molecular basis of ACTs, and we compare transcriptome analysis with other diagnostic approaches. ACTs are occasionally difficult to classify. Therefore, molecular techniques, such as microarray analysis, have recently been applied to overcome some of these diagnostic problems. We also discuss the likelihood of the diagnosis and discernment between ACAs and ACCs based on the molecular tests. To show the recent progress in understanding the etiology of ACTs, we highlight the relationship between genetic analysis and transcriptome analysis. We attempt to understand the role of abnormal cell growth and steroid hormone secretion. Genetic and transcriptome analyses have improved our understanding of ACTs considerably, yet many unanswered questions remain.
  

  PMID: 22559973 [PubMed - in process]

• 

  The use of immunohistochemical expression of SF-1 and EMA in distinguishing adrenocortical tumors from renal neoplasms.

  Fetched: May 8th, 2012, 11:00pm MDT

  The use of immunohistochemical expression of SF-1 and EMA in distinguishing adrenocortical tumors from renal neoplasms.

  Appl Immunohistochem Mol Morphol. 2012 Mar;20(2):141-5

  Authors: Enriquez ML, Lal P, Ziober A, Wang L, Tomaszewski JE, Bing Z

  Abstract
  Steroidogenic factor -1 (SF-1) is an orphan member of the nuclear hormone receptor superfamily, and is considered to play an important role in the differentiation of steroidogenic tissues. In this study, we compared the immunohistochemical stains of SF-1 and epithelial membrane antigen (EMA) in non-neoplastic adrenal tissue, and adrenal and renal tumors using tissue microarrays (TMAs). The adrenal tissue array included 19 cases of normal adrenal cortex, 22 cases of adrenal adenoma, and 20 cases of adrenal cortical carcinoma. The renal tissue array included 20 cases of each of the following types of renal cell carcinoma: clear cell, papillary, and chromophobe. In addition, 20 cases of renal oncocytoma were also included in the study. SF-1 showed positive staining in all cases (100%) of normal adrenal cortex and adrenal cortical adenoma, and in 18 (90%) cases of adrenocortical carcinoma. In renal tumors, SF-1 showed negative stains in all of oncocytoma, papillary, and chromophobe renal cell carcinoma. Only 3 out of 20 cases of clear cell renal cell carcinoma showed weak positivity in approximately 10% of tumor cells. EMA stained positively in 85%, 95%, 100%, and 95% of clear cell, papillary, chromophobe renal cell carcinomas, and oncocytomas, respectively. EMA was completely negative in the adrenal TMAs. In conclusion, SF-1 and EMA may be helpful in the differentiation of adrenal tumors from renal tumors in difficult cases.
  

  PMID: 22553814 [PubMed - in process]

• 

  Combination Chemotherapy in Advanced Adrenocortical Carcinoma.

  Fetched: May 8th, 2012, 11:00pm MDT

  Combination Chemotherapy in Advanced Adrenocortical Carcinoma.

  N Engl J Med. 2012 May 2;

  Authors: Fassnacht M, Terzolo M, Allolio B, Baudin E, Haak H, Berruti A, Welin S, Schade-Brittinger C, Lacroix A, Jarzab B, Sorbye H, Torpy DJ, Stepan V, Schteingart DE, Arlt W, Kroiss M, Leboulleux S, Sperone P, Sundin A, Hermsen I, Hahner S, Willenberg HS, Tabarin A, Quinkler M, de la Fouchardière C, Schlumberger M, Mantero F, Weismann D, Beuschlein F, Gelderblom H, Wilmink H, Sender M, Edgerly M, Kenn W, Fojo T, Müller HH, Skogseid B,

  Abstract
  Background Adrenocortical carcinoma is a rare cancer that has a poor response to cytotoxic treatment. Methods We randomly assigned 304 patients with advanced adrenocortical carcinoma to receive mitotane plus either a combination of etoposide (100 mg per square meter of body-surface area on days 2 to 4), doxorubicin (40 mg per square meter on day 1), and cisplatin (40 mg per square meter on days 3 and 4) (EDP) every 4 weeks or streptozocin (streptozotocin) (1 g on days 1 to 5 in cycle 1; 2 g on day 1 in subsequent cycles) every 3 weeks. Patients with disease progression received the alternative regimen as second-line therapy. The primary end point was overall survival. Results For first-line therapy, patients in the EDP-mitotane group had a significantly higher response rate than those in the streptozocin-mitotane group (23.2% vs. 9.2%, P<0.001) and longer median progression-free survival (5.0 months vs. 2.1 months; hazard ratio, 0.55; 95% confidence interval [CI], 0.43 to 0.69; P<0.001); there was no significant between-group difference in overall survival (14.8 months and 12.0 months, respectively; hazard ratio, 0.79; 95% CI, 0.61 to 1.02; P=0.07). Among the 185 patients who received the alternative regimen as second-line therapy, the median duration of progression-free survival was 5.6 months in the EDP-mitotane group and 2.2 months in the streptozocin-mitotane group. Patients who did not receive the alternative second-line therapy had better overall survival with first-line EDP plus mitotane (17.1 month) than with streptozocin plus mitotane (4.7 months). Rates of serious adverse events did not differ significantly between treatments. Conclusions Rates of response and progression-free survival were significantly better with EDP plus mitotane than with streptozocin plus mitotane as first-line therapy, with similar rates of toxic events, although there was no significant difference in overall survival. (Funded by the Swedish Research Council and others; FIRM-ACT ClinicalTrials.gov number, NCT00094497 .).
  

  PMID: 22551107 [PubMed - as supplied by publisher]

• 

  RIBONUCLEOTIDE REDUCTASE LARGE SUBUNIT (RRM1) GENE EXPRESSION MAY PREDICT EFFICACY OF ADJUVANT MITOTANE IN ADRENOCORTICAL CANCER.

  Fetched: May 4th, 2012, 11:00pm MDT

  RIBONUCLEOTIDE REDUCTASE LARGE SUBUNIT (RRM1) GENE EXPRESSION MAY PREDICT EFFICACY OF ADJUVANT MITOTANE IN ADRENOCORTICAL CANCER.

  Clin Cancer Res. 2012 Apr 30;

  Authors: Volante M, Terzolo M, Fassnacht M, Rapa I, Germano A, Sbiera S, Daffara F, Sperone P, Scagliotti GV, Allolio B, Papotti M, Berruti A

  Abstract
  PURPOSE: Mitotane is the most broadly used systemic therapy for adrenocortical carcinoma (ACC), but its mechanism of action and predictors of treatment response are currently poorly defined. Our aim was to evaluate the gene expression of ribonucleotide reductase large subunit 1 (RRM1) and excision repair cross-complementation group 1 (ERCC1) in ACC as potential biomarkers for clinical outcome and response to mitotane. EXPERIMENTAL DESIGN: Forty-five and 47 tissue samples from two cohorts (Orbassano, Italy; Wuerzburg, Germany) of completely resected ACC were centrally analyzed using Real Time PCR for RRM1 and ERCC1 expression. Fifty-four patients received surgery alone and 38 received adjuvant mitotane after surgery. Clinical and pathological features were highly comparable in the two series. H295R and SW-13 ACC cell lines were also used for pharmacological tests.RESULTS: ERCC1 gene expression was not associated to clinical outcome. In contrast, high RRM1 gene expression was associated to shorter disease-free and overall survival at both univariate and multivariate analysis. In patients with low RRM1 gene expression adjuvant mitotane was associated with improved disease free survival, whereas this effect was lost in cases with high RMM1 expression. In vitro mitotane induced strong up-regulation of RRM1 transcription (up to 25-fold increase) in mitotane-insensitive SW-13 but not in mitotane-sensitive H295R cells. Furthermore, RRM1 silencing in SW-13 cells induced sensitivity to mitotane. CONCLUSIONS: Our in vitro and in vivo data indicate that RRM1 gene expression is functionally associated to mitotane sensitivity and support a possible role of RRM1 determination as a novel molecular biomarker predicting response to adjuvant mitotane in ACC.
  

  PMID: 22547773 [PubMed - as supplied by publisher]

• 

  Mitotane exhibits dual effects on steroidogenic enzymes gene transcription under basal and cAMP-stimulating microenvironments in NCI-H295 cells.

  Fetched: May 4th, 2012, 11:00pm MDT

  Mitotane exhibits dual effects on steroidogenic enzymes gene transcription under basal and cAMP-stimulating microenvironments in NCI-H295 cells.

  Toxicology. 2012 Apr 23;

  Authors: Lin CW, Chang YH, Pu HF

  Abstract
  Adrenocortical carcinoma (ACC) is an extremely rare and aggressive endocrine malignancy with a poor prognosis. The most common symptom of ACC is hypercortisolism (Cushing's syndrome), which has the highest mortality. Mitotane is used as a steroidogenesis inhibitor for Cushing's syndrome or as a chemical adrenalectomy drug for ACC. Mitotane induces adrenal cortex necrosis, mitochondrial membrane impairment, and irreversible binding to CYP proteins. In this study, we explored the molecular effect of mitotane on steroidogenesis in human adrenocortical cancer NCI-H295 cells. Mitotane (10-40μM) inhibited basal and cAMP-induced cortisol secretion but did not cause cell death. Mitotane exhibited an inhibitory effect on the basal expression of StAR and P450scc protein. Furthermore, 40μM of mitotane significantly diminished StAR, CYP11A1 and CYP21 mRNA expression. HSD3B2 and CYP17 seem to be insensitive to mitotane. The stimulatory effects of mitotane on CYP11B1 were more remarkable than its inhibitory effects. In contrast, the activation of cAMP signaling strongly elevated the expression of all these genes. Mitotane (40μM) almost completely neutralized this positive effect and returned 8-Br-cAMP-induced StAR, CYP11A1, CYP17 and CYP21 mRNA to control levels. After cAMP activation, mitotane did not change the levels of CYP11B1 mRNA. The present study demonstrates that mitotane can inhibit cortisol biosynthesis due to a non-specific interference with the gene transcription of steroidogenic enzymes under both basal and 8-Br-cAMP-activated conditions in NCI-H295 cells. We also identified that StAR and CYP11A1 key enzymes that participate in the rate-limiting step of steroidogenesis, were more sensitive to mitotane. In addition, the biphasic effect of mitotane on CYP11B1 was also elucidated.
  

  PMID: 22546480 [PubMed - as supplied by publisher]

• 

  Adrenocortical carcinoma extending into the inferior vena cava in a patient with right kidney agenesis: Surgical approach and review of literature.

  Fetched: May 2nd, 2012, 11:00pm MDT

  Adrenocortical carcinoma extending into the inferior vena cava in a patient with right kidney agenesis: Surgical approach and review of literature.

  Int J Surg Case Rep. 2012 Apr 3;3(7):302-304

  Authors: Meniconi RL, Caronna R, Schiratti M, Dinatale G, Russillo GC, Liguori A, Chirletti P

  Abstract
  INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis and the association with tumor thrombus into the inferior vena cava (IVC) is not common. The best treatment is represented by radical surgery. PRESENTATION OF CASE: We describe a case of a large ACC of the left adrenal gland extending into the IVC through the left renal vein in a young patient with agenesis of the right kidney and signs of acute renal failure. A midline laparotomy was performed, subsequently extended by a left thoracophrenotomy through the 7th intercostal space in order to control the proximal surface of the mass and the thoracic aorta. The tumor was completely excised preserving the kidney, and thrombectomy was performed by a cavotomy with a temporary caval clamping, without cardiopulmonary by-pass (CPB). DISCUSSION: We discuss surgical approaches reported in literature in case of ACC with intracaval extension. The tumor must be completely resected and the thrombectomy can be performed by different approaches: cavotomy with direct suture, partial resection of caval wall without reconstruction, resection of vena cava with graft reconstruction. These procedures could require a CPB, with an increased mortality. In our case we preserved the kidney and a thrombectomy without CPB was performed. CONCLUSION: Intracaval extension of ACC does not represent a contraindication to surgery. The best treatment of intracaval thrombus should be the cavotomy with direct suture. The CPB is not always required. In presence of renal agenesis, the preservation of the kidney is mandatory.
  

  PMID: 22543230 [PubMed - as supplied by publisher]

• 

  An unusual presentation of congenital adrenocortical carcinoma: a case report and review of the literature.

  Fetched: May 2nd, 2012, 11:00pm MDT

  An unusual presentation of congenital adrenocortical carcinoma: a case report and review of the literature.

  Cancer Imaging. 2012;12:118-21

  Authors: Singhal M, Kang M, Khadwal A, Duggal R, Rajwanshi A, Khandelwal N

  Abstract
  We describe a case of congenital non-functional adrenocortical carcinoma in a male infant who presented with recurrent pneumonia, paraparesis and sclerotic skeletal metastasis. To the best of our knowledge such presentation has never been reported.
  

  PMID: 22542918 [PubMed - in process]

• 

  Resection of Adrenocortical Carcinoma Liver Metastasis: Is it Justified?

  Fetched: April 27th, 2012, 11:00pm MDT

  Resection of Adrenocortical Carcinoma Liver Metastasis: Is it Justified?

  Ann Surg Oncol. 2012 Apr 21;

  Authors: Gaujoux S, Al-Ahmadie H, Allen PJ, Gonen M, Shia J, D'Angelica M, Dematteo R, Fong Y, Blumgart L, Jarnagin WR

  Abstract
  BACKGROUND: Adrenocortical carcinoma (ACC) liver metastases (LM) represent a therapeutic challenge, and it is unclear whether resection is justified. This study assesses long-term outcome and prognostic factors after liver resection for metastatic ACC. METHODS: Patients who underwent resection of ACC LM were identified from institutional databases. Recurrence, survival, and tumor characteristics, including β-catenin and TP53 status based on immunohistochemistry and sequencing, were reviewed. The prognostic value of variables was assessed with log-rank test for univariate analysis and Cox proportional hazard models for multivariate analysis. RESULTS: From 1978 to 2009, 28 patients (20 females; median age, 45 years), including 11 with synchronous metastasis and 3 with extrahepatic metastasis, underwent resection for ACC LM (major hepatectomy in 61%). Postoperative mortality was nil and morbidity 55%. On pathological examination, tumors were multiple in 68%, with a median size of 43 mm, and resections were R0, 1, and 2 in 59%, 33%, and 7%, respectively. All 28 patients developed recurrent disease, which was treated surgically in 11, including repeat hepatectomy in 4. Of the 15 patients with adequate tissue for analysis, β-catenin immunostaining was positive in 7, with 4 corresponding CTNNB1 mutations associated with decreased survival; p53 staining was positive in 5 (4 with corresponding TP53 mutations). The median disease-free and overall survival after hepatectomy was 7 and 31.5 months, respectively, with a 5-year survival of 39%. In multivariate analysis, nonfunctional tumor and surgical treatment of recurrence were independent predictors of good outcome. CONCLUSIONS: In selected patients with ACC LM, resection is associated with long-term survival and is, therefore, justified but rarely curative.
  

  PMID: 22526905 [PubMed - as supplied by publisher]

• 

  Worsening Central Sarcopenia and Increasing Intra-Abdominal Fat Correlate with Decreased Survival in Patients with Adrenocortical Carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  Worsening Central Sarcopenia and Increasing Intra-Abdominal Fat Correlate with Decreased Survival in Patients with Adrenocortical Carcinoma.

  World J Surg. 2012 Apr 19;

  Authors: Miller BS, Ignatoski KM, Daignault S, Lindland C, Doherty M, Gauger PG, Hammer GD, Wang SC, Doherty GM,

  Abstract
  BACKGROUND: Accurate prediction of survival from adrenocortical carcinoma (ACC) is difficult and current staging models are unreliable. Central sarcopenia as part of the cachexia syndrome is a marker of frailty and predicts mortality. This study seeks to confirm that psoas muscle density (PMD), lean psoas muscle area (LPMA), lumbar skeletal muscle index (LSMI), and intra-abdominal (IA) or subcutaneous fat (SC) can be used in combination to more accurately predict survival in ACC patients. METHODS: PMD, LPMA, IA, and SC fat were measured on serial CT scans of patients with ACC. Clinical outcome was correlated with quantitative data from patients with ACC and analyzed. A linear regression model was used to describe the relationship between PMD, LPMA, LSMI, IA, and SC fat, time to recurrence, and length of survival according to tumor stage. RESULTS: One hundred twenty-five ACC patients (94 females) were treated from 2005 to 2011. Significant morphometric predictors of survival include PMD, LPMA, and IA fat (p ≤ 0.0001, ≤0.0024, <0.0001, respectively) and improve prediction of survival compared to using stage alone. A 100-mm(2) increase in LPMA confers an 8 % lower hazard of death. LSMI does not change significantly between stages (p = 0.3196). CONCLUSION: Decreased PMD, LPMA, and increased IA fat suggest decreased survival in ACC patients and correlate with traditional staging systems. A more precise prediction of survival may be achieved when staging systems and morphometric measures are used in combination. Serial measurements of morphometric data are possible. The rate of change of these variables over time may be more important than the absolute value.
  

  PMID: 22526034 [PubMed - as supplied by publisher]

• 

  Metronomic chemotherapy may be active in heavily pre-treated patients with metastatic adreno-cortical carcinoma.

  Fetched: April 27th, 2012, 11:00pm MDT

  Metronomic chemotherapy may be active in heavily pre-treated patients with metastatic adreno-cortical carcinoma.

  J Endocrinol Invest. 2012 Apr 5;

  Authors: Ferrero A, Sperone P, Ardito A, Rossi G, Del Buono S, Priola AM, Bracarda S, Taberna E, Terzolo M, Berruti A

  Abstract
  Objective: the potential benefit of further chemotherapy approaches in patients with adrenocortical carcinoma (ACC) showing progressive disease after 2 chemotherapy lines is actually unknown. This study provide explorative information on the activity of metronomic chemotherapy in heavily pretreated ACC patients. Design and methods: we tested the activity of cytotoxic treatments administered on a metronomic schedule in metastatic ACC patients showing disease progression after treatment with gemcitabine and capecitabine scheme. Results: eight patients out of 28 consecutively enrolled in that trial were treated with several metronomic cytotoxic regimens. Six of them showed disease progression but 2 patients obtained a clear benefit. The first patient was treated with oral etoposide (50 mg daily) as the 6th line therapy and obtained a partial response lasting 24 months, while the second patient obtained a partial response lasting 10 months with metronomic oral cyclophosphamide (50 mg daily) as the 5th chemotherapy line. Both patients had sex hormone secreting tumors and were bearing a rather indolent ACC. Conclusions: the administration of several chemotherapy lines in advanced ACC patients cannot be routinely recommended outside of prospective clinical trials. Few patients with indolent tumors, however, may benefit from this approach. According to our experience, oral cyclophosphamide and oral etoposide may be used in this setting.
  

  PMID: 22522572 [PubMed - as supplied by publisher]

• 

  Unusual DNA mismatch repair-deficient tumors in Lynch syndrome: a report of new cases and review of the literature.

  Fetched: April 27th, 2012, 11:00pm MDT

  Unusual DNA mismatch repair-deficient tumors in Lynch syndrome: a report of new cases and review of the literature.

  Hum Pathol. 2012 Apr 17;

  Authors: Karamurzin Y, Zeng Z, Stadler ZK, Zhang L, Ouansafi I, Al-Ahmadie HA, Sempoux C, Saltz LB, Soslow RA, O'Reilly EM, Paty PB, Coit DG, Shia J, Klimstra DS

  Abstract
  Immunohistochemical detection of DNA mismatch repair proteins and polymerase chain reaction detection of microsatellite instability have enhanced the recognition of mismatch repair-deficient neoplasms in patients with Lynch syndrome and, consequently, led to the identification of tumors that have not been included in the currently known Lynch syndrome tumor spectrum. Here, we report 4 such unusual tumors. Three of the 4, a peritoneal mesothelioma, a pancreatic acinar cell carcinoma, and a pancreatic well-differentiated neuroendocrine tumor, represented tumor types that, to the best of our knowledge, have not been previously reported in Lynch syndrome. The fourth tumor was an adrenocortical carcinoma, which has rarely been reported previously in Lynch syndrome. Three of our 4 patients carried a pathogenic germ-line mutation in a mismatch repair gene. The unusual tumor in each of the 3 patients showed loss of the mismatch repair protein corresponding to the mutation. The fourth patient did not have mutation information but had a history of colonic and endometrial carcinomas; both lacked MSH2 and MSH6 proteins. Interestingly, none of the 4 unusual tumors revealed microsatellite instability on polymerase chain reaction testing, whereas an appendiceal carcinoma from 1 of the study patients who was tested simultaneously did. The recognition of such tumors expands the repertoire of usable test samples for the workup of high-risk families. As yet, however, there are no data to support the inclusion of these tumors into general screening guidelines for detecting Lynch syndrome, nor are there data to warrant surveillance for these tumors in patients with Lynch syndrome.
  

  PMID: 22516243 [PubMed - as supplied by publisher]

• 

  Molecular markers of adrenocortical tumors.

  Fetched: April 18th, 2012, 11:00pm MDT

  Molecular markers of adrenocortical tumors.

  J Surg Oncol. 2012 Apr 13;

  Authors: Jain M, Rechache N, Kebebew E

  Abstract
  Adrenocortical tumors are common and incidentally discovered in up to 14% of axial imaging studies performed for other indications. Most of these tumors are nonfunctioning but may require removal because of the risk of adrenocortical carcinoma. Unfortunately, most clinical and imaging features are still not accurate enough to allow definitive diagnosis and an increasing number of patients undergo adrenalectomy to exclude a cancer diagnosis. Adrenocortical carcinoma is an aggressive malignancy with no effective therapy for patients with locally advanced and metastatic disease. Studies using new genomic approaches including mRNA, miRNA, methylation, and CGH profiling have identified dysregulated genes and pathways that may have clinical implications in improved molecular diagnosis and prognostication of adrenocortical cancer (ACC). In this review, we highlight recent advances in the molecular diagnosis of adrenocortical tumors. Published 2012. This article is a U.S. Government work and is in the public domain in the USA.
  

  PMID: 22504887 [PubMed - as supplied by publisher]

• 

  Adrenocortical tumors in children 18 years old and younger.

  Fetched: April 16th, 2012, 11:00pm MDT

  Adrenocortical tumors in children 18 years old and younger.

  J Korean Surg Soc. 2012 Apr;82(4):246-50

  Authors: Cho MJ, Kim DY, Kim SC, Kim TH, Kim IK

  Abstract
  PURPOSE: Pediatric adrenocortical tumors (ACTs) are rare. We reviewed findings in 8 children, 18 years of age or younger, diagnosed with ACT in our institution over the past 15 years.
  METHODS: We retrospectively reviewed 8 children with ACTs treated between 1996 and 2010.
  RESULTS: Three girls and 5 boys were treated for ACTs; their median age at presentation was 144 months (range, 28 months to 18 years). Seven patients showed signs of endocrine dysfunction, 4 with Cushing syndrome, 2 with virilization, and 1 with hyperaldosteronism. One patient, with symptoms of hematuria, underwent a computed tomography scan, which showed an adrenal mass. The median duration of symptoms prior to resection was 6 months (range, 1 to 24 months). Five patients had adenomas and 3 had carcinomas. All underwent complete resection of the tumor, with laparoscopic adrenalectomy performed on 3 patients with adenoma and 1 with carcinoma. The median tumor weight was 12.5 g (range, 1 to 130 g) and the median tumor volume was 18.3 cm(3) (range, 2.2 to 299.2 cm(3)). At a median follow-up of 5.1 years (range, 4 months to 15 years), all 8 patients remain alive with no recurrence of disease.
  CONCLUSION: The characteristics of pediatric ACTs vary considerably. Laboratory findings, clinical hormonal features, and tumor size could not distinguish adenomas from carcinomas before surgery. Complete tumor resection was successful, with no tumor recurrence. However, the small number of patients and short follow-up period limit assessments of prognosis.
  

  PMID: 22493766 [PubMed - in process]

• 

  The role of radiation therapy in the management of adrenal carcinoma and adrenal metastases.

  Fetched: April 16th, 2012, 11:00pm MDT

  The role of radiation therapy in the management of adrenal carcinoma and adrenal metastases.

  J Surg Oncol. 2012 Apr 4;

  Authors: Milgrom SA, Goodman KA

  Abstract
  The use of radiation therapy (RT) to treat adrenal tumors has historically been limited by the risk of normal tissue toxicity, given the proximity of the adrenals to radiosensitive structures, such as the kidney, stomach, intestine, and spinal cord. However, contemporary techniques have made RT safe and effective for use in the management of adrenal carcinoma and adrenal metastases. Data on recent advances in the use of RT to treat adrenocortical carcinoma and adrenal metastases are reviewed, in both surgical and non-surgical settings. J. Surg. Oncol © 2012 Wiley Periodicals, Inc.
  

  PMID: 22488095 [PubMed - as supplied by publisher]

• 

  Surgical Management and Clinical Prognosis of Adrenocortical Carcinoma.

  Fetched: April 16th, 2012, 11:00pm MDT

  Surgical Management and Clinical Prognosis of Adrenocortical Carcinoma.

  Urol Int. 2012 Apr 5;

  Authors: Dong D, Li H, Yan W, Ji Z, Mao Q

  Abstract
  Objective: To study the relationship between surgical management and prognosis of adrenocortical carcinoma (ACC) in order to guide the surgical management of ACC. Methods: Clinical data of 45 cases of ACC treated in our hospital were retrospectively analyzed. The 45 cases included 3 cases in stage I, 12 cases in stage II, 7 cases in stage III, and 23 cases in stage IV. 17 cases underwent complete excision, 14 cases underwent palliative excision, 8 cases had non-operative treatment and 6 cases gave up treatment. All patients were followed up from 2 to 141 months. Results: The average survival time of 31 patients with surgery was 32.46 months, and the average survival time of 14 patients without surgery was 4.75 months. There were statistically significant differences between the two groups (p < 0.01). There were no statistically significant differences between the two groups in survival time in stage III and stage IV (p > 0.05). Conclusions: Surgery is considered to be the only method to cure ACC. For ACC in stage I and II, tumor resection is the most effective treatment, and second surgical operation is recommended for local recurrence. For ACC in stage III, extensive surgical operation is recommended, and for ACC in stage IV, surgical operation has no effect on the prognosis.
  

  PMID: 22487802 [PubMed - as supplied by publisher]

• 

  Adrenal neoplasms.

  Fetched: April 16th, 2012, 11:00pm MDT

  Adrenal neoplasms.

  Clin Radiol. 2012 Apr 7;

  Authors: Low G, Dhliwayo H, Lomas DJ

  Abstract
  Adenoma, myelolipoma, phaeochromocytoma, metastases, adrenocortical carcinoma, neuroblastoma, and lymphoma account for the majority of adrenal neoplasms that are encountered in clinical practice. A variety of imaging methods are available for evaluating adrenal lesions including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine techniques such as meta-iodobenzylguanidine (MIBG) scintigraphy and positron-emission tomography (PET). Lipid-sensitive imaging techniques such as unenhanced CT and chemical shift MRI enable detection and characterization of lipid-rich adenomas based on an unenhanced CT attenuation of ≤10 HU and signal loss on opposed-phase compared to in-phase T1-weighted images, respectively. In indeterminate cases, an adrenal CT washout study may differentiate adenomas (both lipid-rich and lipid-poor) from other adrenal neoplasms based on an absolute percentage washout of >60% and/or a relative percentage washout of >40%. This is based on the principle that adenomas show rapid contrast washout while most other adrenal neoplasms including malignant tumours show slow contrast washout instead. (18)F-2-fluoro-2-deoxy-d-glucose-PET ((18)FDG-PET) imaging may differentiate benign from malignant adrenal neoplasms by demonstrating high tracer uptake in malignant neoplasms based on the increased glucose utilization and metabolic activity found in most of these malignancies. In this review, the multi-modality imaging appearances of adrenal neoplasms are discussed and illustrated. Key imaging findings that facilitate lesion characterization and differentiation are emphasized. Awareness of these imaging findings is essential for improving diagnostic confidence and for reducing misinterpretation errors.
  

  PMID: 22486993 [PubMed - as supplied by publisher]

• 

  [Adrenocortical carcinoma detected by retroperitoneal hemorrhage : a case report].

  Fetched: April 16th, 2012, 11:00pm MDT

  [Adrenocortical carcinoma detected by retroperitoneal hemorrhage : a case report].

  Hinyokika Kiyo. 2012 Mar;58(3):149-53

  Authors: Sakata R, Tsuchiya F, Osaka K, Fujikawa A, Ouchi H, Iwasaki A

  Abstract
  Spontaneous massive retroperitoneal hemorrhage from an adrenal tumor is rare and is usually fatal if unrecognized. We report a case of spontaneous rupture of a primary adrenocortical carcinoma that occurred in a 79-year-old man. He visited our hospital with left abdominal pain. Computed tomography (CT) showed a left retroperitoneal hemorrhage. We could not find the origin of this hemorrhage. Two months later, CT showed the left adrenal tumor, and left adrenalectomy and nephrectomy were performed successfully. The histological diagnosis was adrenocortical carcinoma. He rejected adjuvant therapy. Local recurrence of the tumor was found, and right adrenal gland, brain, and mediastinal lymph node metastases were recognized 6 months after the operation. He died 11 months after the operation.
  

  PMID: 22495043 [PubMed - in process]

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  Oncocytic adrenal cortical carcinosarcoma with pleomorphic rhabdomyosarcomatous metastases.

  Fetched: April 11th, 2012, 11:00pm MDT

  Oncocytic adrenal cortical carcinosarcoma with pleomorphic rhabdomyosarcomatous metastases.

  Am J Surg Pathol. 2012 Mar;36(3):470-7

  Authors: Thway K, Olmos D, Shah C, Flora R, Shipley J, Fisher C

  Abstract
  Adrenal cortical carcinosarcoma is a rare variant of adrenal cortical carcinoma. Sarcomatous change in adrenal cortical carcinomas is exceptionally rare, with only 9 cases previously described. Adrenal cortical carcinosarcomas tend to be aggressive tumors, with locoregional recurrence and rapid metastases from the sarcomatoid component. We describe what seems to be the first case of sarcoma arising in oncocytic adrenal cortical carcinoma. The sarcomatous component here was pleomorphic rhabdomyosarcoma. This occurred in a 45-year-old man who had nodal and pulmonary metastases of the rhabdomyosarcomatous component at presentation and who died of progressive disease 11 months later. Here, we discuss the clinical, radiologic, and pathologic findings and review the literature on adrenal cortical carcinosarcomas.
  

  PMID: 22343339 [PubMed - indexed for MEDLINE]

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  Adrenocortical cancer in Carney complex: a paradigm of endocrine tumor progression or an association of genetic predisposing factors?

  Fetched: April 11th, 2012, 11:00pm MDT

  Adrenocortical cancer in Carney complex: a paradigm of endocrine tumor progression or an association of genetic predisposing factors?

  J Clin Endocrinol Metab. 2012 Feb;97(2):387-90

  Authors: Bertherat J

  PMID: 22312093 [PubMed - indexed for MEDLINE]

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  A large family with Carney complex caused by the S147G PRKAR1A mutation shows a unique spectrum of disease including adrenocortical cancer.

  Fetched: April 11th, 2012, 11:00pm MDT

  A large family with Carney complex caused by the S147G PRKAR1A mutation shows a unique spectrum of disease including adrenocortical cancer.

  J Clin Endocrinol Metab. 2012 Feb;97(2):351-9

  Authors: Anselmo J, Medeiros S, Carneiro V, Greene E, Levy I, Nesterova M, Lyssikatos C, Horvath A, Carney JA, Stratakis CA

  Abstract
  CONTEXT: Most tumors in Carney complex (CNC) are benign, including primary pigmented nodular adrenocortical disease (PPNAD), the main endocrine tumor in CNC. Adrenocortical cancer (AC) has never been observed in the syndrome. Herein, we describe a large Azorean family with CNC caused by a point mutation in the PRKAR1A gene coding for type 1-α (RIα) regulatory subunit of the cAMP-dependent protein kinase A, in which the index patient presented with AC.
  OBJECTIVE: We studied the genotype-phenotype correlation in CNC.
  DESIGN AND SETTING: We reported on case series and in vitro testing of the PRKAR1A mutation in a tertiary care referral center.
  PATIENTS: Twenty-two members of a family were investigated for Cushing syndrome and other CNC components; their DNA was sequenced for PRKAR1A mutations.
  RESULTS: Cushing syndrome due to PPNAD occurred in four patients, including the proposita who presented with AC and three who had Cushing syndrome and/or PPNAD. Lentigines were found in six additional patients who did not have PPNAD. A base substitution (c.439A>G/p.S147G) in PRKAR1A was identified in the proposita, in the three others with PPNAD, in the proposita's twin daughters who had lentigines but no evidence of hypercortisolism, and in five other family members, including one without lentigines or evidence of hypercortisolism. Unlike in other RIα defects, loss of heterozygosity was not observed in AC. The S147G mutation was compared to other expressed PRKAR1A mutations; it led to decreased cAMP and catalytic subunit binding by RIα and increased protein kinase A activity in vitro.
  CONCLUSIONS: In a large family with CNC, one amino acid substitution caused a spectrum of adrenal disease that ranged from lack of manifestations to cancer. PPNAD and AC were the only manifestations of CNC in these patients, in addition to lentigines. These data have implications for counseling patients with CNC and are significant in documenting the first case of AC in the context of PPNAD.
  

  PMID: 22112814 [PubMed - indexed for MEDLINE]

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  Clinical and pathophysiological implications of chromosomal alterations in adrenocortical tumors: an integrated genomic approach.

  Fetched: April 11th, 2012, 11:00pm MDT

  Clinical and pathophysiological implications of chromosomal alterations in adrenocortical tumors: an integrated genomic approach.

  J Clin Endocrinol Metab. 2012 Feb;97(2):E301-11

  Authors: Barreau O, de Reynies A, Wilmot-Roussel H, Guillaud-Bataille M, Auzan C, René-Corail F, Tissier F, Dousset B, Bertagna X, Bertherat J, Clauser E, Assié G

  Abstract
  PURPOSE: Diagnosing malignancy of adrenocortical tumors (ACT) and predicting prognosis in carcinomas are often challenging. Transcriptome markers have recently emerged, providing promising clinical relevance and improved pathophysiological knowledge. Whether tumoral chromosomal alterations provide similar information is not known. The aim was to evaluate the diagnostic and prognostic value of chromosomal alterations in ACT and to identify genes associated with benign and malignant tumorigenesis.
  EXPERIMENTAL DESIGN: Chromosomal alterations of 86 adenomas and 52 carcinomas were identified by comparative genomic hybridization arrays and/or quantitative PCR.
  RESULTS: A larger proportion of the genome is altered in carcinomas compared with adenomas (44 vs. 10%, P = 2.10(-10)). In adenomas, the 9q34 region, which includes the steroidogenic factor 1 locus, is commonly gained and associated with an overexpression of steroidogenic factor 1 (SF-1). In carcinomas, recurrent gains include chromosomes 5, 7, 12, 16, 19, and 20 and recurrent losses chromosomes 13 and 22. Filtering the genes from these regions according to their expression profile identified genes potentially relevant to adrenocortical tumorigenesis. A diagnostic tool was built by combining DNA copy number estimates at six loci (5q, 7p, 11p, 13q, 16q, and 22q). This tool discriminates carcinomas from adenomas in an independent validation cohort (sensitivity 100%, specificity 83%). In carcinomas, the number of chromosomal alterations was not associated with survival (Cox P = 0.84). A prognostic tool based on tumor DNA was designed with a clustering strategy and validated in an independent cohort.
  CONCLUSIONS: Chromosomal alterations in ACT discriminate carcinomas from adenomas and contain prognostic information. Chromosomal alterations alter the expression of genes important for tumorigenesis.
  

  PMID: 22112813 [PubMed - indexed for MEDLINE]

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  Genetics of adrenocortical disease: an update.

  Fetched: April 7th, 2012, 11:00pm MDT

  Genetics of adrenocortical disease: an update.

  Curr Opin Endocrinol Diabetes Obes. 2012 Apr 3;

  Authors: Bar-Lev A, Annes JP

  Abstract
  PURPOSE OF REVIEW: Disease states characterized by abnormal cellular function or proliferation frequently reflect aberrant genetic information. By revealing disease-specific DNA mutations, we gain insight into normal physiology, pathophysiology, potential therapeutic targets and are better equipped to evaluate an individual's disease risks. This review examines recent advances in our understanding of the genetic basis of adrenal cortical disease. RECENT FINDINGS: Important advances made in the past year have included identification of KCNJ5 potassium channel mutations in the pathogenesis of both aldosterone-producing adenomas and familial hyperaldosteronism type III; characterization of phosphodiesterase 11A as a modifier of phenotype in Carney complex caused by protein kinase, cAMP-dependent, regulatory subunit, type-I mutations; the finding of 11β-hydroxysteroid dehydrogenase type I mutations as a novel mechanism for cortisone reductase deficiency; and demonstration of potential mortality benefit in pursuing comprehensive presymptomatic screening for patients with Li-Fraumeni syndrome, including possible reduction in risks associated with adrenocortical carcinoma. SUMMARY: This research review provides a framework for the endocrinologist to maintain an up-to-date understanding of adrenal cortical disease genetics.
  

  PMID: 22476103 [PubMed - as supplied by publisher]

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  Adrenocortical carcinoma: Past, present, and future.

  Fetched: April 7th, 2012, 11:00pm MDT

  Adrenocortical carcinoma: Past, present, and future.

  J Surg Oncol. 2012 Apr 3;

  Authors: Lafemina J, Brennan MF

  Abstract
  Adrenocortical carcinoma (ACC) is a rare endocrine malignancy. Due to its rarity, heterogeneity, and a lack of a comprehensive understanding of the pathogenesis, little progress has been made in treatment and outcomes. The current review explores the past, present, and future of the understanding and treatment of this disease process. J. Surg. Oncol © 2012 Wiley Periodicals, Inc.
  

  PMID: 22473597 [PubMed - as supplied by publisher]

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  DNA Methylation Profiling Identifies Global Methylation Differences and Markers of Adrenocortical Tumors.

  Fetched: April 7th, 2012, 11:00pm MDT

  DNA Methylation Profiling Identifies Global Methylation Differences and Markers of Adrenocortical Tumors.

  J Clin Endocrinol Metab. 2012 Apr 3;

  Authors: Rechache NS, Wang Y, Stevenson HS, Killian JK, Edelman DC, Merino M, Zhang L, Nilubol N, Stratakis CA, Meltzer PS, Kebebew E

  Abstract
  Context:It is not known whether there are any DNA methylation alterations in adrenocortical tumors.Objective:The objective of the study was to determine the methylation profile of normal adrenal cortex and benign and malignant adrenocortical tumors.Methods:Genome-wide methylation status of CpG regions were determined in normal (n = 19), benign (n = 48), primary malignant (n = 8), and metastatic malignant (n = 12) adrenocortical tissue samples. An integrated analysis of genome-wide methylation and mRNA expression in benign vs. malignant adrenocortical tissue samples was also performed.Results:Methylation profiling revealed the following: 1) that methylation patterns were distinctly different and could distinguish normal, benign, primary malignant, and metastatic tissue samples; 2) that malignant samples have global hypomethylation; and 3) that the methylation of CpG regions are different in benign adrenocortical tumors by functional status. Normal compared with benign samples had the least amount of methylation differences, whereas normal compared with primary and metastatic adrenocortical carcinoma samples had the greatest variability in methylation (adjusted P ≤ 0.01). Of 215 down-regulated genes (≥2-fold, adjusted P ≤ 0.05) in malignant primary adrenocortical tumor samples, 52 of these genes were also hypermethylated.Conclusions:Malignant adrenocortical tumors are globally hypomethylated as compared with normal and benign tumors. Methylation profile differences may accurately distinguish between primary benign and malignant adrenocortical tumors. Several differentially methylated sites are associated with genes known to be dysregulated in malignant adrenocortical tumors.
  

  PMID: 22472567 [PubMed - as supplied by publisher]

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  Clinical and imaging overview of functional adrenal neoplasms.

  Fetched: April 7th, 2012, 11:00pm MDT

  Clinical and imaging overview of functional adrenal neoplasms.

  Int J Urol. 2012 Mar 29;

  Authors: Low G, Sahi K

  Abstract
  Adrenal adenoma, adrenocortical carcinoma, pheochromocytoma and neuroblastoma are four discrete adrenal neoplasms that have the potential for functional activity. Functional adrenal neoplasms can secrete cortisol, aldosterone, sex hormones or catecholamines. These heterogeneous groups of tumors show varied biological behavior and clinical outcomes. These neoplasms are encountered with increasing clinical frequency as a result of an expansion in the volume of medical imaging carried out. The clinical presentation, including prognosis and treatment options, and the imaging features of these neoplasms are discussed. The key radiological observations of each of these neoplasms are shown using multimodality images. Familiarity with the clinical and imaging features of these neoplasms improves diagnosis, and facilitates appropriate clinical decision-making and patient management.
  

  PMID: 22462796 [PubMed - as supplied by publisher]

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  Primary adrenal gland carcinosarcoma associated with metastatic rectal cancer: a hitherto unreported collision tumor.

  Fetched: April 7th, 2012, 11:00pm MDT

  Primary adrenal gland carcinosarcoma associated with metastatic rectal cancer: a hitherto unreported collision tumor.

  Tumori. 2011 Sep-Oct;97(5):27e-30e

  Authors: Bertolini F, Rossi G, Fiocchi F, Giacometti M, Fontana A, Gibertini MC, Roncucci L, Luppi G, Torricelli P, Rossi A, Conte PF

  Abstract
  In this report we describe the case of a young woman with familial adenomatous polyposis who developed metastatic rectal cancer during pregnancy. At diagnosis, we decided to perform a transabdominal laparoscopic adrenalectomy, because of the high risk of bowel obstruction, and to define the origin of the adrenal gland lesion, suspected to be primary on the basis of imaging results. The histological specimen showed a collision tumor between an adrenal metastasis of a rectal tumor and a primary adrenal gland carcinosarcoma. The peculiarity of the case is due not only to its clinical presentation during pregnancy, but also to the presence of this uncommon adrenal collision tumor. A particular challenge for the clinician is to define the priority between these two tumors: the presence of two distinct and colliding aggressive neoplasms poses a problem in the choice of the best therapeutic approach, also given the impossibility to biopsy all metastatic sites. However, we decided to treat the patient as having a metastatic rectal cancer, because we had a solid histological confirmation of metastases.
  

  PMID: 22158506 [PubMed - indexed for MEDLINE]