Last revised by Ashesh Ishwarlal Ranchod on 16 Jul 2023

Ependymomas represent a relatively broad group of glial tumors most often arising from the lining of the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all pediatric brain tumors and up to 33% of brain tumors occurring in those less than 3 years of age. 

Ependymal tumors can occur anywhere within the neuraxis, but distribution and molecular characteristics are fairly site-dependent, divided into three groups depending on the anatomical compartment in which they are found 11,13,15:

  • posterior fossa (60%)
  • supratentorial (30%)
  • spinal cord (10%)

In addition to subependymomas, that can be encountered in all three compartments, DNA-methylation profiling divides ependymomas on the basis of their molecular characteristics and their location as differing in epidemiology and prognosis. As such, location forms the basis of the current (2021) WHO classification 11,13:

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Cases and figures

  • Figure 1: ependymal rosettes
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  • Case 1
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  • Figure 2: EMA positive perinuclear dot
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  • Case 2: supratentorial
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  • Case 3: posterior fossa ependymoma
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