Primary ovarian carcinoid tumors were reviewed from Mayo Clinic and Colorado Tumor Registry data. A total of 17 patients with this diagnosis were identified. Histologic analysis of these carcinoid tumors revealed 9 (53%) were insular, 5 (29%) were trabecular, and 4 (26%) were strumal carcinoid with or without a mature dermoid component. There were 11 patients with stage I and 6 patients with stage III or IV disease at diagnosis. Carcinoid syndrome was found in 29% of patients. Pressure or pain with defecation was noted in 41% of cases. Recurrence of tumor occurred in 1 of 11 patients with suspected stage I disease 13 years after initial diagnosis. Overall survival was excellent in the 11 patients whose disease was confined to one ovary (100% 5-year survival), but only 1 of 6 patients survived (33% 5-year survival) if advanced stage at diagnosis. Systemic chemotherapy for advanced disease achieved a complete response in 1 patient and stable disease in another. Although rare, primary ovarian carcinoid tumors treated with surgery alone and found to be confined to the ovary can be expected to have an excellent overall outcome.
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