Case Report

A Case Series of 27 Primary Ovarian Neuroendocrine Tumors

Author: Michail Shafir

Objective: Primary ovarian neuroendocrine tumors are rare and are often found incidentally. We examined 27 primary ovarian carcinoids treated at a single institution over the course of 21 years; one of the largest series in the literature. The purpose of the study is to report on a large series of infrequent tumors and to assess the clinic-pathological correlations. Material and Methods: All patients with ovarian neuroendocrine tumors between 1994 and 2015 were retrospectively reviewed in this IRB approved chart-review cohort study. No patient was identified or contacted. Patients were identified from various sources, including surgical, pathologic, radiologic, and hospital databases. Pathologic review confirmed 27 primary ovarian carcinoids. Clinical outcomes, surgical and pathological findings were reviewed and analyzed. Results: The mean age of our cohort was 48.7 years (range 23-75 years). The majority of tumors were found to be benign (23/27). Nineteen patients (70.4%) had carcinoids associated with dermoid cysts, of which 9 were associated with thyroid tissue (strumal carcinoid). Seven patients (25.9%) had ovarian mucinous tumors, of which 4 were benign and 3 were malignant. One patient was diagnosed with malignant carcinoid, insular type. Bilateral disease was found in 2 patients (these were diagnosed with a malignant tumor, and at the time of this study, they were both deceased). All patients with benign tumors had no evidence of disease at time of review. Of the 4 malignant tumors, one is alive with disease, two have died of disease, and one was lost to follow up. The 5-year overall survival for patients with benign disease was 86%. Conclusion: Our case series is one of the largest reported. The average age of these patients was younger than previously reported. Most of the carcinoids were associated with teratomas and the majority of the malignant tumors were of the mucinous variant.