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Background Neuroendocrine tumors of the ovary are uncommon malignant carcinomas with a poor prognosis. There are no standardized management guidelines because of lack of knowledge about these tumors. The objective of this study is to analyze the clinical manifestations, diagnosis and treatment of ovarian neuroendocrine tumors (ONETs). Results Median age at diagnosis was 38.4 years (range 20–58). The main clinical presentations were abdominal pain or a pelvic mass. Two patients underwent fertility-sparing surgery and the others underwent cytoreductive surgery. The finial diagnosis depended on postoperative histopathology. According to the staging standard of the International Federation of Gynecology and Obstetrics (FIGO), three patients had stage I disease, one stage II, and six patients had stage III disease. Eight patients received chemotherapy alone postoperatively, one was maintained with Olaparib after chemotherapy, and one received chemotherapy followed by radiotherapy. The follow-up time for patients raged from 10 to 48 months. One patient died, while the other 9 patients were in follow-up. Seven patients experienced tumor recurrence. Conclusion Ovarian neuroendocrine tumors are characterized by high malignancy, low incidence and poor prognosis. Histopathological analysis is considered the gold standard for diagnosis. Surgical resection may be the first choice of therapy, and adjuvant chemotherapy and possible radiotherapy may prolong the survival of some patients.

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.