Dysembryoplastic neuroepithelial tumor

Orpha code: 251946OMIM code:

Definition

A rare mixed neuronal-glial tumor characterized by a benign, usually supratentorial lesion with predominantly cortical location and multinodular architecture. The tumor typically becomes symptomatic in the second or third decade of life with drug-resistant partial seizures. Histological hallmark is the specific glioneuronal element, columns oriented perpendicularly to the cortical surface, formed by bundles of axons attached to oligodendroglia-like cells, while neurons appear to float in an abundant eosinophilic matrix.

Disease data
Classification

Disease

Synonyms
DNET
DNET
ORPHA code
251946
OMIM code
-
ICD10 code
D33.0
ICD11 code
2A00.21

No additional description.

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