Ovarian Neuroendocrine Tumors: a Single-institution Experience

Background: Ovarian neuroendocrine tumors (O-NETs) are uncommon malignant carcinomas with a poor prognosis. There are no standardized practice guidelines as impacted by the lack of sufficient data regarding the clinicopathologic features and management of O-NETs. This study aimed to analyze the clinical manifestations, diagnosis and treatment of O-NETs. Methods: : We retrospectively analyzed ten cases with ONETs admitted to the Department of Gynecology of the First Affiliated Hospital of Zhengzhou University from August, 2015 to May, 2020. Results: The median age of cases at diagnosis was 38.4 years (from 20 to 58). The most common presentation was abdominal pain (4/10), followed by pelvic mass (3/10), and vaginal bleeding (1/10). Two cases had no symptoms. All cases underwent the surgery. The diagnosis was confirmed by the postoperative histopathology. Tumors expressed at least one neuroendocrine marker (e.g., CD56, chromogranin-A, synaptophysin, or neuron specific enolase). By complying with the International Federation of Gynecology and Obstetrics (FIGO) staging system (2014), three cases were stage I tumors, one case was stage II tumor, and six cases were stage III tumors. Eight cases only received chemotherapy postoperatively, one was administrated with olaparib maintenance after chemotherapy, and one received the chemotherapy followed by the radiotherapy. The follow-up time for cases raged from 10 to 48 months. Two cases died of disease recurrence, while the other 8 cases were in the follow-up. Six cases experienced tumor recurrence. Conclusion: O-NETs were characterized by high malignancy, low incidence and poor prognosis. The histopathological analysis was considered the gold standard for diagnosis. Surgical resection might be the first choice of therapy, and adjuvant chemotherapy and possible radiotherapy might prolong the survival of some cases.