SDPS-14 CLINICAL FEATURES AND OUTCOME IN CHILDREN WITH CHOROID PLEXUS CARCINOMA-A SINGLE CENTER EXPERIENCE

OBJECTIVE: To investigate the clinical features, treatment and outcome in pediatric choroid plexus carcinoma (CPC) patients. METHODS: Twelve children with CPC confirmed by pathology who were admitted to Beijing Shijitan Hospital from January 2017 to October 2022, were included. The last follow-up was December 31, 2022. Kaplan-Meier method was adopted for survival analysis. RESULTS: Among the 12 patients, 4 were males and 8 females. The median age at diagnosis was 29.7 (range, 5.8-119.6) months, with 8 cases less than 3 years old at the time of diagnosis. Nine cases were supratentorial, and 3 infratentorial. The tumor was located in ventricular system in 6 cases, external involvement of brain parenchyma in 6 cases. Two cases experienced metastases (M+) and 10 M0 at the time of diagnosis. All patients received tumor resection, with 8 cases GTR and 4 NTR. Five cases received chemotherapy only and 7 patients radio-chemotherapy after surgery. At last follow-up, 8 cases experienced tumor recurrence or progression, and 4 died. The mean OS were (56.7±8.8) months, The 1, 3, 5-year OS were (83.3%±10.8)%、(66.7%±13.6)%, and (66.7%±13.6)%, respectively. The mean PFS time were (24.3±7.2) months, The 1, 3-year PFS were (41.7%±14.2)% and (33.3%±13.6)%, respectively. Univariate analysis showed the OS were poorer in children with infratentorial tumors than supratentorial tumors (χ2 =8.562, P = 0.003). And the OS was also lower in patients who received chemotherapy only than those who received both radiotherapy and chemotherapy (χ2 =8.488, P =0.004). The difference in PFS between groups were not significant (P>0.05). CONCLUSION: CPC is very rare and with poor prognosis in children. Children with infratentorial tumors and who received chemotherapy only tend to have poorer OS. Due to the small sample size, further clinical trials need to be developed to verify.