Intracranial choroid plexus carcinomas: Report of 11 cases from a single institution.

Abstract Background Choroid plexus carcinoma is a central nervous system tumor pathologically corresponding to World Health Organization grade III. Choroid plexus carcinoma mainly affects pediatric patients with a poor prognosis. Due to its rarity, the standardized treatment has not yet been outlined. Methods We retrospectively analyzed 11 patients with histopathologically diagnosed choroid plexus carcinoma between January 2008 and December 2016. They were treated with surgical resection with or without adjuvant therapies. The clinical profiles and outcomes were analyzed. Results The mean age at diagnosis was 16.0 years (median, 7.0 years; range, 4 months ∼ 59 years). Gross total resection was achieved in 9 cases, and subtotal resection in 2 cases. Seven patients received adjuvant radiotherapy, and two patients underwent chemotherapy. The mean overall survival was 34.8 months, and the mean progression-free survival was 24.5 months. During the follow-up period, four patients succumbed to central nervous system dissemination of choroid plexus carcinoma, including two patients with malignant transformation from atypical choroid plexus papilloma to choroid plexus carcinoma and one patient treated with the combined chemotherapy protocol. Conclusions In this study, we described the clinicoradiological characteristics of choroid plexus carcinomas. Surgical resection is the mainstream treatment. Due to the paucity of clinical evidence, the standard regimen of adjuvant therapies still needs further research.