ABSTRACT

Ependymal tumors represent a diverse collection of brain neoplasms with a multitude of clinical presentations. This chapter will analyze the salient clinical features necessary for the clinician to successfully diagnose and manage ependymoma and anaplastic ependymoma, intramedullary ependymoma, myxopapillary ependymoma, and subependymoma. These neoplasms are of neuroectodermal origin and arise from the neuroepithelial lining of the ventricles, central canal of the spinal cord, choroid plexus, white matter adjacent to a ventricular surface, and filum terminale. They are classified by the World Health Organization (WHO) as subependymoma (grade I), ependymoma (grade II), anaplastic ependymoma (grade III), and myxopapillary ependymoma (grade I). The focus of this review will be on epidemiology, specific clinical features, therapy, and prognosis, as detailed reviews of pathology and radiology are addressed elsewhere. The unifying theme among the ependymal tumors is that they all require surgical resection as the exclusive therapy or as part of it.