Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature

Am J Surg Pathol. 1997 Jul;21(7):763-71. doi: 10.1097/00000478-199707000-00004.

Abstract

Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a rare recently described entity. Only three examples have been documented, one of which showed evidence of malignant transformation. We report an additional four cases and update the literature. With the exception of an 82-year-old man, all patients have been under 30 years of age. The temporal lobe was involved in three cases and cerebellum in another three. Radiologic features were those common to PXA and GG. Histologically, all were "collision tumors" composed of abutting, although spatially distinct, PXA and GG components. In two cases, the second element was only recognized at reexcision or recurrence. Histologic anaplasia, always in the PXA component, was evident as brisk mitotic activity and/or necrosis in five cases. Of the seven patients, one died of disease 17 years after the onset of seizures and after multiple recurrences, the last of which largely resembled glioblastoma. We conclude that the composite PXA-GG is a rare neoplasm that shares many features of its individual components. In addition to its temporal lobe predilection, the cerebellum is frequently affected. As when it occurs in isolation, the PXA component of composite PXA-GG possesses the potential for malignant transformation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Astrocytes / pathology
  • Astrocytoma / diagnostic imaging
  • Astrocytoma / pathology*
  • Brain Neoplasms / diagnostic imaging
  • Brain Neoplasms / pathology*
  • Cell Nucleus / pathology
  • Cerebellar Neoplasms / diagnostic imaging
  • Cerebellar Neoplasms / pathology
  • Cytoplasm / pathology
  • Fatal Outcome
  • Female
  • Ganglioglioma / diagnostic imaging
  • Ganglioglioma / pathology*
  • Glial Fibrillary Acidic Protein / analysis
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Recurrence, Local / pathology
  • S100 Proteins / analysis
  • Tomography, X-Ray Computed

Substances

  • Glial Fibrillary Acidic Protein
  • S100 Proteins