RELA fusion-positive ependymoma

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RELA fusion-positive ependymoma

Synonyms: Supratentorial C11ORF95-RELA fused 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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version September 2023.

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RELA fusion-positive ependymoma?

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