Ependymoma

Roger J Packer MD (Dr. Packer of George Washington University; Senior Vice President, Center for Neuroscience and Behavioral Medicine; and Gilbert Endowed Distinguished Professor in Neurofibromatosis and Director, Gilbert Neurofibromatosis Institute and Brain Tumor Institute, Children’s National Health System, has no relevant financial relationships to disclose.)
Originally released August 8, 1994; last updated July 25, 2017; expires July 25, 2020

This article includes discussion of ependymoma, benign ependymomas, clear cell ependymomas, malignant ependymomas, anaplastic ependymomas, myxopapillary ependymomas, subependymomas, and ependymomas. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Ependymomas are 1 of the more common childhood brain tumors. After total resection, outcome is excellent. However, for patients whose tumors are subtotally resected or anaplastic, disease relapse is common. The author summarizes the outcomes of new treatment approaches and the possible value of adjuvant chemotherapy. Increased biological understandings have already changed classification and likely, in the near future, will dramatically alter management of the tumor. They have clearly demonstrated that pediatric supratentorial and infratentorial ependymomas are different entities.

Historical note and terminology

Ependymomas were first described by Bailey in 1924. Initial classification schema using light microscopy findings subdivided ependymomas into epithelial, myxopapillary, and cellular types (Bailey 1924). Because of the variable relationship demonstrated between histological findings and outcome in different subgroupings of ependymomas, other classification schemas have been proposed.

The most recent World Health Organization classification of CNS tumors defines ependymoma as a tumor composed predominantly of neoplastic ependymal cells (Louis et al 2007). This classification system distinguishes ependymoma WHO grade II from anaplastic or malignant ependymoma, WHO grade III. Myxopapillary ependymomas are considered a distinct variant of ependymoma occurring almost exclusively in the region of the cauda equina (WHO grade I). Subclassifications of ependymoma include cellular ependymoma, papillary ependymoma, and RELA-fusion positive ependymoma. The latter is a new entity, incorporating molecular aspects into classification for the first time (Louis et al 2016). It is likely that further molecularly-based subclassification will be utilized in the future (Pajtler et al 2017). Subependymomas are nodular tumors that are thought to have a different, more benign prognosis than other types of ependymomas that occur in the brain.

Ependymoblastoma was initially considered a subtype of ependymoma. Histologically, ependymoblastomas are primarily composed of small, undifferentiated cells associated with regions of relatively well-formed ependymal blastic rosettes. The tumor is now classified as an embryonal tumor and is primarily considered a subtype of primitive neuroectodermal tumor of childhood. In the revised World Health Organization classification of CNS tumors, ependymoblastomas are given a separate designation under the general category of embryonal tumors. Others do not even consider ependymomas a distinct entity (Judkins and Ellison 2008).

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