Pediatric ependymomas: Something old, something new

Abstract

Ependymoma is one of the most common central nervous system (CNS) tumors in childhood. Ependymoma had previously been considered a single disease entity that arises in the posterior fossa, supratentorial compartment, or spinal cord; however recent advances in our understanding of the molecular make-up of this tumor reveal it to be not one disease, but many. In addition, the behavior of the disease and outcomes for children stricken with it are associated with the molecular abnormalities noted. In this update, we will review what is known about the molecular classification of ependymoma, the treatments that have been proven to work (namely surgery and radiation), the role of chemotherapy, newer agents being investigated, and the impact of molecular characteristics on strategies to design clinical trials and improve outcomes for children with ependymoma.

Keywords

• Ependymoma
• Pediatric
• Brain and spinal cord tumors