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Primary ovarian carcinoids are usually unilateral. In about 15% of cases, the contralateral ovary has a mature cystic teratoma or a mucinous cystadenoma. If the tumor is admixed with small foci of teratoma, it is likely to be a primary ovarian tumor. The prognosis is excellent.

Metastatic carcinoid tumors to the ovary are almost always bilateral and show scattered tumor deposits throughout both ovaries. Other features supporting metastases include: peritoneal and extraovarian involvement, lymphovascular invasion, and association with carcinoid syndrome. Carcinoid syndrome may persist despite removal of the ovarian tumor. The most common primary sites (for insular pattern) are ileum, duodenum, jejunum, and lung. If the ovarian tumor has mucinous or goblet cell appearance, the most likely primary site is the appendix. Prognosis is poor.

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