RELA fusion-positive ependymoma

What is RELA fusion-positive ependymoma?

A rare ependymal tumor characterized by the presence of a RELA fusion gene. This supratentorial grade II or III ependymoma most often occurs in children and young adults. Histopathological features are variable, but a distinctive vascular pattern of branching capillaries or clear-cell change are common. Patients may present with focal neurological deficits, seizures, or features of raised intracranial pressure. Prognosis is worse than in other supratentorial ependymomas.

Other condition names

  • Supratentorial C11ORF95-RELA fused ependymoma

Age of Onset

  • Adolescent
  • Adult
  • Childhood
  • Infancy
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