Papillary glioneuronal tumor

Orpha code: 251962OMIM code:

Definition

A rare mixed neuronal-glial tumor characterized by a supratentorial space-occupying lesion in periventricular location, often with prominent cystic change. The histological hallmark of this low-grade neoplasm is its pseudopapillary appearance with a single layer of cuboidal cells around hyalinized blood vessels, associated with sheets or focal collections of neuronal cells. Clinical presentation is variable and non-specific, most frequently with headache and seizures. Prognosis is favorable after complete resection.

Disease data
Classification

Disease

Synonyms
PGNT
PGNT
Pseudopapillary ganglioglioneurocytoma
Pseudopapillary neurocytoma with glial differentiation
Pseudopapillary ganglioglioneurocytoma
Pseudopapillary neurocytoma with glial differentiation
ORPHA code
251962
OMIM code
-
ICD10 code
D33.0
ICD11 code
2A00.21

No additional description.

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