Pleomorphic xanthoastrocytoma

Orpha code: 251607OMIM code:

Definition

A rare low-grade astrocytoma characterized by superficial location in the cerebral hemispheres with involvement of the meninges, composed of GFAP-expressing cells showing nuclear and cytoplasmic pleomorphism and xanthomatous change, surrounded by a reticulin network. The tumor corresponds to WHO grade II and typically affects children and young adults, who often present with a long history of seizures. Extent of resection and mitotic index are important prognostic factors.

Disease data
Classification

Disease

Synonyms
PXA
PXA
ORPHA code
251607
OMIM code
-
ICD10 code
C71.9
ICD11 code
2A00.0Y

No additional description.

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