||
RELA-fusie-positief supratentoriëel ependymoom (aandoening)
RELA-fusie-positief supratentoriëel ependymoom
RELA-fusie-positief ependymoom van supratentoriële hersenen
supratentoriëel C11ORF95-RELA-gefuseerd ependymoom
RELA fusion-positive supratentorial 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.
Id1217383007
StatusDefined
SNOMED CT to Orphanet simple map530792
SNOMED CT to ICD-10 extended map
TargetC71.0
RuleTRUE
AdviceALWAYS C71.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified