LINC-17. A single center retrospective analysis of pediatric intracranial choroid plexus carcinoma in Beijing

Objective: To explore the clinical characteristics and outcome in children with intracranial choroid plexus carcinoma in Beijing. Methods: We retrospectively analyzed 13 children with pathologically confirmed diagnosis of choroid plexus carcinoma between January 2017 and December 2021. They were treated with surgical resection followed by adjuvant therapies. Results: Male/female ratio=0.625(5:8). The median age at diagnosis was 1.2 years (range, 6 months to 9.8 years) with 9 cases < 3 years of age. Gross total resection was achieved in 12 cases (92.3%), and subtotal resection in 1 case. Seven patients received only adjuvant chemotherapy according to CPT-SIOP-2009 protocol, and 6 patients underwent radiotherapy with combined chemotherapy. Six patients developed a recurrence, and 4 cases died at last follow-up. Among 9 patients alive, 7 cases attained CR, 1 PR and 1 SD.The 5-year progression-free survival (PFS) and overall survival (OS) were (64.8±14.3) % and (68.4 ± 13.1) %, respectively. The survival of children treated under both irradiation and chemotherapy were higher than chemotherapy only (P<0.05). The cases < 3 years of age had worse survival than those ≥3 years (P<0.05), and the 4 cases died were all younger than 3 years old. Conclusions: Choroid plexus carcinoma is a rare and malignant brain tumor, and affects mainly younger children in our center. Surgical resection is the mainstream treatment. Adjuvant radiotherapy with combined chemotherapy and patients older than 3 years old tend to have better survival. Keywords: Choroid plexus carcinoma; Therapy; Survival.