UCSF

Choroid plexus carcinoma (CPC) is a rare, malignant, primary brain tumor with a poor prognosis. While previous reports have shown benefits of aggressive surgery, very few large-scale studies have focused exclusively on the pediatric population, for whom the risks of aggressive surgery must be weighed carefully against the benefits. We performed a comprehensive systematic review of pediatric CPCs to test the effects of gross total resection (GTR) on overall survival (OS) and progression-free survival (PFS). A Pubmed search was performed to identify children with CPC who underwent surgical resection. Only disaggregated clinical cases in which extent of resection was confirmed by CT or MRI were included for analysis. Kaplan-Meier and multivariate Cox regression survival analyses were performed to determine the effects of extent of resection on OS and PFS. Disaggregated clinical data from a total of 102 pediatric CPC patients (age ≤18 years) with known extent of resection and overall survival were analyzed. GTR was significantly associated with better OS by Kaplan-Meier analysis (logrank p < 0.001). Multivariate Cox regression analysis adjusting for age, gender, tumor location (supratentorial vs. infratentorial), and type of adjuvant therapy (chemotherapy, radiation, and combined therapy), showed that GTR independently increased OS (p = 0.006). While GTR also improved PFS on Kaplan-Meier analysis (p = 0.027), the effect did not meet our criteria for significance in our multivariate Cox model (p = 0.120). GTR improved OS of pediatric CPC and is recommended if it can be safely performed.