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Appendix Cancer

Name  l  Define  l  Risk  l  Detect  l  Treatment  l  Stage/Grade  l  Followup  l  Mets/Recur  l  Link

Names - Synonyms

Appendix cancer, appendiceal cancer, see definition below for types


Appendiceal cancers are considered malignancies of the gastrointestinal system, accounting for about 1 in 200 gastrointestinal malignancies. The appendix (aka: vermiform process) is part of that system, although it has no known function or purpose in present day man. 

Note: If you are seeking research for your appendiceal cancer in a book, the first place to look is the gastrointestinal section. You may not find anything because of it's rarity. Between 1973 and 1998, 2117 appendiceal malignancies were reported to the United States SEER program.  That's less than 85 cases a year!

There are a number of non-cancerous tumors or nodules of the appendix; including adenoma (serrated, tubular, tubulovillous, villous), benign mesothelioma, cystadenoma (cystic adenoma), granular cell tumor, leiomyoma, lipoma, mucinous adenoma, mucinous cystadenoma (low grade appendiceal mucinous neoplasm), neurofibroma, neuroma, and polyp.

Borderline tumors, which may be malignant of benign, include fibrous histiocytic tumor, mucinous neoplasm of low malignant potential ( most commonly associated with pseudomyxoma peritonei), and mucinous neoplasm of uncertain malignant potential.

Malignancies of the appendix include adenocarcinoma, gastrointestinal stromal tumor (very rare), kaposi sarcoma, leiomyosarcoma, mixed cell (carcinoid & adenocarcinoma), mucinous adenocarcinoma (carcinoma), non-Hodgkin lymphoma (very rare), signet ring cell adenocarcinoma (carcinoma), small cell carcinoma, and undifferentiated carcinoma.  Appendiceal carcinoids include endocrine/neuroendocrine {enterochromaffin (EC) (serotonin producing); non-argentaffin (L-Cell) (glucagon-like peptide)}, goblet cell carcinoma (adenocarcinoid), and tubular.

Conditions or syndromes that may coexist with a diagnosis of appendiceal tumor include disseminated peritoneal adenomucinosis, peritoneal carcinomatosis, and pseudomxoma peritonei

Risk Factors - Causes

Some familial risks have been reported for appendiceal carcinoids, such as the MEN1 syndrome.  Gastritis, pernicious anemia, or Zollinger-Ellison syndrome may also predispose a person to carcinoid.  Age may be a factor.


Intussusception, palpable mass, gastrointestinal bleeding, increasing abdominal girth, or secondary genitourinary complications may all be signs of an appendiceal tumor.  Redness or a feeling of warmth in the face and neck, diarrhea, shortness of breath, fast heartbeat, tiredness, swelling of the feet and ankles, wheezing, pain or a feeling of fullness in the abdomen could be indicative of a carcinod or neuroendocrine tumor.

Blood tests, blood chemistry studies, and 24 hour urine tests might be used to rule out a carcinoid.  Tumors that infiltrate large portions of the appendix may be seen on CT scan.  Somatostatin receptor scintigraphy (SRS) may be used to diagnose carcinoids of the appendix.

Staging may include endoscopy, biopsy, xray, SRS, angiogram, PET and/or CT scans.  Some appendix cancers are diagnosed after removal of the appendix for acute appendicitis.  Sadly, many cancers of the appendix are diagnosed at autopsy.


Treatment for cancers of the appendix are normally based on the involvement of the cancer.  Localized, smaller, tumors are normally treated with surgery, alone.  Appendiceal cancer that has moved out of the appendix may be treated with surgical procedures, cytoreductive surgery, and/or intraperitoneal hyperthermia.  

Surgical techniques; such as ablation, fulguration, cryosurgery, ligation, or embolization; and  radiotherapy, chemotherapy, and chemoembolization may also be used for carcinoids.  Ethanol injection, hormone therapy, and biologic therapies might be used in conjunction with other treatments.

Stage - Grade

Carcinoid tumors are usually slow growing.  Carcinoids are staged according to their involvement:
Localized - Encapsulated with the appendix.
Regional - In other areas of the gastrointestinal tract, lymph nodes, or surrounding tissue.
Metastatic - In areas outside of the gastrointestinal system.

Suggested Followups

Metastasis - Recurrence


Appendix Cancer (Appendiceal) Support Forum