pleomorphic xanthoastrocytoma (PXA)

Epidemiology

• characteristically occurs in children or young adults
• accounts for < 1% of all astrocytic neoplasms

Pathology

• gross pathology
  • usually more well demarcated
  • tends to be superficially located in cerebral hemispheres
  • temporal lobe most common location
  • may be cystic
• tumor characteristics:
  • corresponds histologically to WHO grade 2
  • minority progress to anaplastic astrocytoma or glioblastoma.
  • mitotic index & extent of resection appeared to correlate with tumor recurrence & overall survival
  • Ref proposes the term PXA with anaplastic features for > 5 mitoses per 10 high power fields Microscopic pathology
• pleomorphic cells
• mono- or multi-nucleated giant cells sometimes containing lipid vacuoles
• granular bodies, eosinophilic or pale
• reticulin staining around individual/clusters of tumor cells
• GFAP varies, often intense
• nuclear inclusions
• occasionally Rosenthal fibers
• mitotic figures &/or necrosis may be present

Immunophenotype

• GFAP positive most cells
• beta tubulin

Clinical manifestations

• seizure

Radiology

• large well circumscribed mass with cystic and solid components or cyst with mural nodule

Management

Prognosis

• generally favorable prognosis
• 70% ten year survival rate

More general terms

• astrocytoma (astrocytic neoplasm)