Childhood Ependymoma Treatment (PDQ®)–Health Professional Version

General Information About Childhood Ependymoma

Primary brain tumors, including ependymomas, are a diverse group of diseases that together constitute the most common solid tumor of childhood. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification. Brain tumors are classified according to histology, but tumor location, extent of spread, molecular features, and age are important factors that affect treatment and prognosis.

According to the 2016 revision to the World Health Organization (WHO) classification of tumors of the central nervous system, ependymal tumors are classified into the following five main subtypes:[1]

Subependymoma (WHO grade I).
Myxopapillary ependymoma (WHO grade I).
Ependymoma (WHO grade II).
Ependymoma, RELA fusion–positive (WHO grade II or grade III).
Anaplastic ependymoma (WHO grade III).

The PDQ childhood brain tumor treatment summaries are organized primarily according to the WHO classification of nervous system tumors.[1] For a full description of the classification of nervous system tumors and a link to the corresponding treatment summary for each type of brain tumor, refer to the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview.