Prolonged disease-free survival with immunotherapy combined with chemotherapy in a patient with advanced stage large cell neuroendocrine carcinoma of the uterus

Background: Large Cell Neuroendocrine Carcinoma (LCNEC) is a rare and aggressive high-grade neuroendocrine malignancy that only infrequently arises in the uterus. Owing to its rarity, there are no standardized treatment guidelines, and management is generally extrapolated from neuroendocrine carcinomas of the lung or cervix. Data on the role of immune checkpoint inhibition in uterine LCNEC are lacking. We report, to our knowledge, the first case of mismatch repair (MMR)-deficient endometrial LCNEC treated with multimodal therapy including adjuvant chemotherapy, radiation, and maintenance immunotherapy, resulting in prolonged disease-free survival. Case Presentation: -aortic regions. At 29 months post-operation, she remains on maintenance immunotherapy with excellent functional status and no radiographic evidence of disease. Conclusions: This case illustrates that aggressive multimodal therapy including immune checkpoint inhibition may achieve durable disease control in advanced-stage MMR-deficient uterine LCNEC. While the relative contributions of surgery, chemotherapy, radiation, and immunotherapy cannot be separated, the favorable outcome supports further investigation of immunotherapy in rare high-grade endometrial neuroendocrine carcinomas and underscores the importance of including these histologies in clinical trials.