Pediatric ependymoma: A single-center experience from a developing country

Indian J Cancer. Jul-Sep 2021;58(3):378-386. doi: 10.4103/ijc.IJC_373_19.

Abstract

Background: Ependymomas are the third most common pediatric central nervous system (CNS) tumors, accounting for 6-12% of brain tumors in children. Management of these tumors remains challenging and recurrence occurs in over 50% of cases, mainly when complete resection is not achieved before radiotherapy. The 5-year overall survival (OS) ranges from 39 to 64%, with a 5-year progression-free survival (PFS) rate of 23-45%. The study aimed to describe the OS and PFS rates of cases of pediatric ependymoma. It also aimed to evaluate the effects of different variables on disease outcomes. Variables examined included patient age, the extent of surgical resection, radiotherapy and chemotherapy delivered, the histopathological subtype of the tumor, primary tumor location, and extent of the disease at presentation. Last, the challenges that potentially compromise treatment outcomes in resource-limited countries were to be highlighted.

Methods: This is a retrospective cohort study, representing a single-center experience that included 47 pediatric patients treated at the National Cancer Institute, Cairo University, between January 2009 and December 2014.

Results: Median follow-up stood at 23.5 months (range: 2-77 months). The average 3-year OS and PFS rates were 43.7 and 43.3%, respectively.

Conclusion: The extent of surgical excision (maximal resection) and the adequacy of postoperative radiotherapy were the only two factors that had significantly affected the outcome. Understandably, treatment outcomes for ependymomas in developing countries still lag behind best reported outcomes, mainly due to inadequate surgical excision and postoperative radiotherapy.

Keywords: Childhood cancers; developing countries; ependymoma; radiation therapy; surgery..