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Aim  The aim of the present study was to summarize the clinical characteristics and analyze the independent prognostic factors in patients with high‐grade endometrial neuroendocrine carcinoma (ENC).    Methods  Patients diagnosed with ENC, endometrioid adenocarcinoma (EAC), endometrial clear‐cell carcinoma (ECC), endometrial serous carcinoma (ESC), endometrioid carcinoma with mucinous features (EMC) from 1987 to 2016 were screened from the National Cancer Institute database (surveillance, epidemiology, and end results [SEER]). Kaplan–Meier were used to assess survival. Univariate and multivariate Cox proportional hazards analysis were done to examine factors affecting survival.    Results  The median survival times of ENC were 11 months, shorter than that of EAC, ECC, ESC, and EMC ( p  < 0.01). There was no significant difference in ages, survival rate, and median survival time between large‐cell ENC (LCENC) and small‐cell ENC (SCENC), which were all belong to ENC. In a multivariable model, the hazard ratio (HR) of death for women with Federation International of Gynecology and Obstetrics (FIGO) stage I‐II of ENC was 0.37 compared to FIGO stage III‐IV ( p  < 0.01). The HR of patients who under the surgery was 0.39 compared to the patients who without surgery ( p  < 0.01), and the HR of patients who received chemotherapy was 0.51 compared to the patients who did not received chemotherapy ( p  < 0.01). Radiotherapy did not significantly reduce the mortality risk of patients.    Conclusion  ENC was a kind of devastating endometrial cancers with the poorest prognosis. Surgical treatment and chemotherapy were necessary for improving prognosis of ENC. Early diagnosis favored better prognosis. There was no prognostic difference between with and without radiotherapy.

Clinical characteristic and prognostic factors in high‐grade endometrial neuroendocrine carcinoma