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PubMed - Adrenocortical Carcinoma (35 unread)

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]
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]
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]
[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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]

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