Gastrointestinal stromal tumors are rare tumors with malignant potential. Primary lymphomas can occur in the appendix. Breast cancer, colon cancer, and tumors of the female genital tract may metastasize to the appendix.
The treatment for tumors varies. Small carcinoids (<2 cm) without features of malignancy may be treated by appendectomy if complete removal is possible. Other carcinoids and adenocarcinomas may require right hemicolectomy. Note: the term "carcinoids" is outdated: these tumors are now more accurately called "neuroendocrine tumors." For more information, see "appendiceal neuroendocrine tumors."
Pseudomyxoma peritonei treatment includes cytoreductive surgery which includes the removal of visible tumor and affected essential organs within the abdomen and pelvis. The peritoneal cavity is infused with heated chemotherapy known as HIPEC in an attempt to eradicate residual disease. The surgery may or may not be preceded or followed with intravenous chemotherapy or HIPEC.
A study of primary malignacies in the United States found a rate of 0.12 cases per 1,000,000 population per year. Carcinoids that were not identified as malignant were not included in this data. Carcinoid is found in roughly 1 in 300 appendectomies for acute appendicitis. A Hong Kong case series of 1492 appendectomies identified 17 neoplasms. Eight were carcinoids without specification of malignant features. Three cases of adenocarcinoma, and one each of cystadenocarcinoma, psedomyxoma peritoneii, and metastasic carcinoma were identified. The remaining tumors were benign. It was previously believed that carcinoid tumors are the most common tumors of the appendix but now data from the SEER program show that mucinous adenocarcinomas are more common.
Actress Audrey Hepburn was diagnosed with appendix cancer, and she died of the disease in 1993.
Serbian musician Vlada Divljan was diagnosed with the cancer in 2012, and he died of subsequent complications in 2015.
- Hamilton, S.R.; Aaltonen, L.A., eds. (2000). "Ch. 5 Tumours of the Appendix". World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System (PDF). Lyon: International Agency for Research on Cancer.
- McGory, Marcia L.; Maggard, Melinda A.; Kang, Hakjung; O'Connell, Jessica B.; et al. (2005). "Malignancies of the appendix: Beyond case series reports". Diseases of the Colon & Rectum 48 (12): 2264–71. doi:10.1007/s10350-005-0196-4. PMID 16258711.
- O'Donnell, M.E.; Carson, J.; Garstin, W.I.H. (2006). "Surgical treatment of malignant carcinoid tumours of the appendix". International Journal of Clinical Practice 61 (3): 431–7. doi:10.1111/j.1742-1241.2006.00875.x. PMID 16911574 – via Medscape.
- Goede, A.C.; Caplin, M.E.; Winslet, M.C. (2003). "Carcinoid tumour of the appendix". British Journal of Surgery 90 (11): 1317–22. doi:10.1002/bjs.4375. PMID 14598408.
- Stinner, B.; Rothmund, M. (2005). "Neuroendocrine tumours (carcinoids) of the appendix". Best Practice & Research: Clinical Gastroenterology 19 (5): 729–38. doi:10.1016/j.bpg.2005.06.003. PMID 16253897.
- Cancer.Net: Appendix Cancer