Ependymomas in Children and Adults

EpendymomasEpendymomas account for approximately 5% of all CNS tumors in adults and around 10% in the pediatric population. Contrary to traditional theories supporting that ependymomas arise from ependymal cellsEpendymal cell, recent studies propose radial glial cellsGlial cells as the cells of origin. In adults, half of the ependymomasEpendymomas arise in the spinal cordSpinal cord, whereas in the pediatric population, almost 90% of ependymomas are located intracranially. Most of the ependymomas are usually low-grade tumors except anaplasticAnaplastic variants and some cases ofRELA-fusion RELA-fusion-positiveRELA-fusion positive ependymomasEpendymomas, a molecular variant consisting the most recent addition to the 2016 World Health OrganizationWorld Health Organization (WHO) (WHO) classification. Of note, the recently described molecular classification of ependymomasEpendymomas into nine distinct subgroups appears to be of greater clinical utility and prognostic value compared to the traditional histopathological classification, and parts of it are expected to be adopted by the WHOWorld Health Organization (WHO) in the near future. Clinical manifestations depend on the location of the tumor with infratentorialInfratentorial ependymomas presenting with acute hydrocephalusHydrocephalus. Gross total resectionGross total resection (GTR) should be the goal of treatment. The prognostic factorsPrognostic factor of patients with ependymomasEpendymomas include age, grade, and location of the tumor, with children with intracranialIntracranial, anaplastic ependymomasAnaplastic ependymomas having the worst prognosisPrognosis. In general, the 5-year overall survivalSurvival of patients with ependymomasEpendymomas is around 60–70%.