At the time of diagnosis, 58% to 64% of patients with carcinoids of the small intestine have metastatic disease in the regional lymph nodes or the liver. Early surgical treatment should include removal of the mesentery by wedge resection and resection of lymph node metastases surrounding the mesenteric artery and vein to preserve intestinal vascular supply and to limit the intestinal resection. With grossly radical tumor resections, patients may remain symptom free for extended periods of time; however, because of the tenacity of carcinoid tumors, patients should undergo lifelong surveillance.
Surgical treatment for advanced carcinoids involves prophylactic removal of mesenteric metastases early on because later the disease may become impossible to manage surgically. Repeat surgery may be necessary if mesenteric metastases are left during primary surgery or have progressed after primary surgery. These operations are difficult because of fibrosis between regions of the intestine, and surgery may result in fistulation, intestinal devascularization, or creation of a short bowel. In patients with inoperable metastatic disease, 5-year survival is approximately 50% for those with inoperable liver metastases and approximately 40% for those with inoperable liver and mesenteric metastases.[4,5]
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
The most common sites of metastasis for pheochromocytoma or extra-adrenal paraganglioma are lymph nodes, bones, lungs, and liver. Patients with known or suspected...
The effect of octreotide (long-acting repeatable, 30 mg intramuscularly every 28 days) on time to tumor progression in patients with metastatic midgut neuroendocrine tumors has been tested in a randomized, placebo-controlled clinical trial. Although the planned study accrual was 162 patients, because of slow accrual, it was stopped after 85 evaluable patients were enrolled. At an interim analysis, the median time to tumor progression was 14.3 months in the octreotide group versus 6 months in the placebo group (HR = 0.34; 95% CI, 0.20-0.59; P < .0001). Quality of life was similar in both treatment groups. There was no difference in overall survival, but about three-quarters of the control group received octreotide at disease progression.[Level of evidence: 1iDiii].
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.
Modlin IM, Lye KD, Kidd M: A 5-decade analysis of 13,715 carcinoid tumors. Cancer 97 (4): 934-59, 2003.
Akerstr�m G, Hellman P: Surgery on neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 21 (1): 87-109, 2007.
Makridis C, Rastad J, Oberg K, et al.: Progression of metastases and symptom improvement from laparotomy in midgut carcinoid tumors. World J Surg 20 (7): 900-6; discussion 907, 1996.
Makridis C, Ekbom A, Bring J, et al.: Survival and daily physical activity in patients treated for advanced midgut carcinoid tumors. Surgery 122 (6): 1075-82, 1997.
Hellman P, Lundstr�m T, Ohrvall U, et al.: Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases. World J Surg 26 (8): 991-7, 2002.
Rinke A, M�ller HH, Schade-Brittinger C, et al.: Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol 27 (28): 4656-63, 2009.