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Pleomorphic xanthoastrocytoma

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • PXA
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: C71.9
  • ICD-11: 2A00.0Y  XH99U2
  • OMIM: -
  • UMLS: C0334586
  • MeSH: -
  • GARD: 10631
  • MedDRA: -

Detailed information


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