RELA fusion-positive ependymoma

Orpha code: 530792OMIM code:

Definicja

A rare ependymal tumor characterized by the presence of a <i>RELA</i> 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.

Disease data
Klasyfikacja

Disease

Synonimy
Supratentorial C11ORF95-RELA fused ependymoma
Supratentorial C11ORF95-RELA fused ependymoma
Kod ORPHA
530792
Kod OMIM
-
Kod ICD10
C71.0
Kod ICD11
-

No additional description.

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