• Arise from neuroendocrine cells throughout the gut and produce endocrine and vasocative substances
• Metastases more likely in tumors > 2 cm
• Most are small and asymptomatic
• Hepatic metastases produce the carcinoid syndrome—cutaneous flushing, diarrhea, bronchoconstriction
• Resection of primary and isolated hepatic metastases indicated for cure or palliation
• Arise from gut enterochromaffin cells
• Part of amine precursor uptake decarboxylase (APUD) system: Describes related neuroendocrine cells
• Midgut carcinoids produce serotonin, substance P, neuromedin A, bradykinin, dopamine, and histamine
• 85% of GI carcinoid tumors are in the appendix; 15% are in the small intestine, 90% of which are in the ileum
• Multiple synchronous tumors are present in 40% of cases
• At the time of surgical diagnosis, 40% of tumors have invaded the muscularis and 45% have metastasized to lymph nodes or liver
• 20% of patients have a second noncarcinoid tumor—breast, lung, and colon
• Mesenteric desmoplastic reaction and mesenteric vascular sclerosis is thought to be secondary to serotonin secretion and results in obstruction, bowel ischemia, and obstruction
• Biologically active substances secreted by carcinoids are usually inactivated in the liver, but hepatic metastases or primary ovarian or bronchial carcinoids release these compounds directly into the systemic circulation, where they produce symptoms
• Chemotherapy (streptozocin, 5-fluorouracil, doxorubicin) may be beneficial for metastatic disease if progressive
• Octreotide and histamine antagonists for carcinoid syndrome
• Intestinal ischemia
• Carcinoid syndrome
• 70% overall 5-year survival rate
• 14-year median survival from time of diagnosis
• 8-year median survival from onset of the carcinoid ...
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