Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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 Classification Disease Synonyms Supratentorial C11ORF95-RELA fused ependymoma Supratentorial C11ORF95-RELA fused ependymoma ORPHA code 530792 OMIM code - ICD10 code C71.0 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl