Rosette-forming glioneuronal tumor

Orpha code: 251975OMIM code:

Definition

Rosette-forming glioneuronal tumor is a rare mixed neuronal-glial tumor characterized by the presence of uniform, rosette- (or pseudorosette-) forming neurocytes with an astrocytic component, together creating a biphasic pattern. It can present with signs of raised intracranial pressure (headache, vomiting, papilledema), hydrocephalus, seizures, ataxia and visual disturbances, or can be diagnosed incidentally in asymptomatic patients. The tumor usually arises in the midline, involving the fourth ventricle or the cerebellum.

Disease data
Classification

Disease

Synonyms
RGNT
DNT móżdżku
Dysembrioplastyczny guz neuroepitelialny móżdżku
RGNT
ORPHA code
251975
OMIM code
-
ICD10 code
D33.1
ICD11 code
2A00.21

No additional description.

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