Molecular Classification and Treatment Strategies for Pediatric Ependymomas

Abstract

  Ependymomas are the third most common brain tumors in children, after medulloblastomas and astrocytoma. Recent advances in an understanding of the molecular biological processes of the brain has allowed for molecular classification of ependymomas along with histological classification. Ependymomas with similar histological types can exhibit completely different molecular biological characteristics depending on the site of occurrence, such as supratentorial, infratentorial, or spinal cord. And these have different prognosis. The primary therapeutic approach for these tumors involves complete surgical resection followed by radiation therapy. However, the efficacy of chemotherapy is unclear. An unfavorable prognosis is noted in cases where gross total resection is not achieved, often leading to local recurrence or dissemination of the tumor.

  Currently, chemotherapy represents pre- and post-operative supplementary treatment. Nevertheless, other novel molecular discoveries and functions can be of significance in substituting the current ependymoma treatment.

  This review includes the 2021 WHO molecular classification of ependymomas and an overview of treatment strategies and recent findings related to pediatric ependymomas.