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Related Experiment Videos

Anaplastic pleomorphic xanthoastrocytoma

C Bayindir1, N Balak, A Karasu

  • 1Department of Neurosurgery, University of Istanbul, School of Medicine, Turkey.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|January 1, 1997
PubMed
Summary
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This case report details an anaplastic pleomorphic xanthoastrocytoma (PXA) with necrosis, a rare finding. Necrosis in PXA indicates a poor prognosis and suggests exclusion from the PXA classification.

Area of Science:

  • Neuro-oncology
  • Pediatric Pathology
  • Surgical Pathology

Background:

  • Pleomorphic xanthoastrocytoma (PXA) is typically a low-grade glial tumor with a favorable prognosis.
  • Histological hallmarks include GFAP-positive cells, bizarre giant cells, and lack of necrosis or vascular proliferation.
  • Anaplastic variants are rare and associated with poorer outcomes.

Observation:

  • A 9-year-old girl presented with an anaplastic pleomorphic xanthoastrocytoma (PXA).
  • The tumor exhibited extensive necrosis, an uncommon feature for PXA.
  • Cerebrospinal fluid (CSF) dissemination was observed, indicating aggressive disease progression.

Findings:

  • Histopathological analysis revealed features consistent with anaplastic PXA (WHO grade 3).

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  • The presence of necrosis, both focal and extensive, was a significant finding.
  • Immunohistochemical analysis supported the anaplastic nature of the tumor.

    • Implications:

    • Necrosis in PXA is a critical indicator of poor prognosis and aggressive behavior.
    • This finding challenges the current classification, suggesting tumors with necrosis should be excluded from the PXA category.
    • Re-evaluation of diagnostic criteria for PXA may be warranted based on this case.