Insular carcinoid primary in the ovary. A clinicopathologic analysis of 48 cases

Forty-eight cases of primary insular carcinoid of the ovary were analyzed from a clinicopathologic viewpoint. Sixteen (33%) were associated with preoperative clinical evidence of the carcinoid syndrome. At operation only one ovary was usually enlarged, but in 16% the contralateral ovary was also enlarged by either a dermoid cyst or a mucinous cystadenoma or cystadenocarcinoma. The volume of the carcinoid was the most important determinant of whether the carcinoid syndrome was present. No patient had the syndrome whose carcinoid formed only a small portion of a teratoma. Pure tumors or components of teratomas between 4 and 7 cm in diameter were associated with the syndrome in one-half, and larger carcinoids in two-third of the cases. Prominent acinar differentiation also correlated with the presence of the syndrome. Although the prognosis was nearly always favorable after the removal of the tumor, tricuspid valve damage continued to progress and led to cardiac decompensation in one patient; fatal recurrences developed in two others. The primary insular carcinoid should be distinguished from carcinoid metastatic to the ovary, which is nearly always bilateral, is usually associated with the presence of peritoneal metastases, and has a poor prognosis.