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Gastrointestinal Carcinoid Tumors Treatment (PDQ®)

Rectal Carcinoids

In general, rectal carcinoids often present as very small, isolated lesions.[1] The TNM system is used for rectal carcinoids, but size appears to be one of the best estimates of recurrence. Rectal carcinoids should be evaluated by endoscopic ultrasound (EUS) or rectal magnetic resonance imaging (MRI). Tumors smaller than 1 cm can be safely removed by endoscopic excision.[2-5] Excised specimens should be examined histologically to exclude muscularis invasion.[2,6-8]. A report about the rectal carcinoid patients in the Surveillance, Epidemiology,and End Results (SEER) database demonstrated that the 5-year survival rate for patients with stage I carcinoid was 97%.[9]

For patients with tumors that are greater than 2 cm or that have invasion of the muscularis as seen by EUS or MRI, surgical resection with abdominoperineal resection (APR) or low anterior resection (LAR) is recommended because of the high rate of nodal metastases and risk of distant metastatic disease. In the report from the SEER database, patients with stage II or III rectal carcinoids had 5-year survival rates of 84% and 20%, respectively.[9] In a report from the National Cancer Database, among 3,287 patients with rectal carcinoids, the 5-year survival rates for patients with stage II or III disease was 87.3% and 35.5%, respectively.[10]

There is considerable debate about whether local excision or rectal resection (i.e., APR or LAR) is needed for tumors that are 1 cm to 2 cm. Although it may be possible to recognize tumors with particular atypia and high mitotic index before embarking on the more radical surgery, the presence of muscularis invasion or regional metastases generally supports rectal resection. In a multicenter series of 100 patients who underwent anterior resection for rectal carcinoids, the rate of nodal metastases for patients with tumors between 1 cm and 2 cm was 31%.[11] In this series, tumor size greater than 1 cm and lymphovascular invasion were the two strongest predictors of lymph node metastases. In patients with distant metastases, prognosis is generally poor, with an overall 5-year survival rate of approximately 30%.[12]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

  1. Soga J: Carcinoids of the rectum: an evaluation of 1271 reported cases. Surg Today 27 (2): 112-9, 1997. [PUBMED Abstract]
  2. Koura AN, Giacco GG, Curley SA, et al.: Carcinoid tumors of the rectum: effect of size, histopathology, and surgical treatment on metastasis free survival. Cancer 79 (7): 1294-8, 1997. [PUBMED Abstract]
  3. Kwaan MR, Goldberg JE, Bleday R: Rectal carcinoid tumors: review of results after endoscopic and surgical therapy. Arch Surg 143 (5): 471-5, 2008. [PUBMED Abstract]
  4. Mani S, Modlin IM, Ballantyne G, et al.: Carcinoids of the rectum. J Am Coll Surg 179 (2): 231-48, 1994. [PUBMED Abstract]
  5. Caplin M, Sundin A, Nillson O, et al.: ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: colorectal neuroendocrine neoplasms. Neuroendocrinology 95 (2): 88-97, 2012. [PUBMED Abstract]
  6. Suzuki H, Ikeda K: Endoscopic mucosal resection and full thickness resection with complete defect closure for early gastrointestinal malignancies. Endoscopy 33 (5): 437-9, 2001. [PUBMED Abstract]
  7. Vogelsang H, Siewert JR: Endocrine tumours of the hindgut. Best Pract Res Clin Gastroenterol 19 (5): 739-51, 2005. [PUBMED Abstract]
  8. Akerström G, Hellman P, Hessman O: Gastrointestinal carcinoids. In: Lennard TWJ, ed.: Endocrine Surgery. 4th ed. Philadelphia, Pa: WB Saunders Ltd, 2009, pp 147-76.
  9. Landry CS, Brock G, Scoggins CR, et al.: A proposed staging system for rectal carcinoid tumors based on an analysis of 4701 patients. Surgery 144 (3): 460-6, 2008. [PUBMED Abstract]
  10. Chagpar R, Chiang YJ, Xing Y, et al.: Neuroendocrine tumors of the colon and rectum: prognostic relevance and comparative performance of current staging systems. Ann Surg Oncol 20 (4): 1170-8, 2013. [PUBMED Abstract]
  11. Shields CJ, Tiret E, Winter DC, et al.: Carcinoid tumors of the rectum: a multi-institutional international collaboration. Ann Surg 252 (5): 750-5, 2010. [PUBMED Abstract]
  12. Akerström G, Hellman P: Surgery on neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 21 (1): 87-109, 2007. [PUBMED Abstract]
  • Updated: July 31, 2014