Cerebral pleomorphic xanthoastrocytoma associated with NF1: an updated review with a rare atypical case from Africa

Neurosurg Rev. 2012 Jul;35(3):313-9; discussion 319. doi: 10.1007/s10143-011-0362-1. Epub 2011 Oct 22.


The occurrence of cerebral pleomorphic xanthoastrocytoma (PXA) in individuals with neurofibromatosis type 1 (NF1) is very rare. We present a 10-year-old Nigerian boy with NF1 who was found to harbor a thalamic-lateral ventricular solid mass lesion whose histologic and immunohistochemical findings were in keeping with PXA. We also carried out an updated review of the PXA-NF1 literature and found only eight previous reports of this clinical disease association. These reports have been limited to only certain regions of the world, with none yet reported from Africa, South America, Australia, and Eastern Europe. As far as we know, this might be the first such report from Africa. The case we present, in addition, demonstrated some other unique clinical, radiological, and histopathologic characteristics which have been highlighted in this review.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Astrocytoma / complications
  • Astrocytoma / diagnosis
  • Astrocytoma / pathology*
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / pathology*
  • Cafe-au-Lait Spots / etiology
  • Humans
  • Male
  • Neurofibromatosis 1 / complications
  • Neurofibromatosis 1 / pathology*
  • Nigeria
  • Tomography, X-Ray Computed