Pleomorphic xanthoastrocytoma: Report of two cases with unconventional clinical presentations

M. Adelita Vizcaíno, Dario V. Caccamo, Ellen Fox, Fausto J. Rodriguez

Research output: Contribution to journalArticlepeer-review

Abstract

Aims: Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic neoplasm with a relative circumscribed architecture that typically arises superficially in the cerebral hemispheres of teenagers and young adults. Our aim is to highlight unconventional clinical presentations of this distinct neoplasm. Materials and methods: We report two cases of PXA with unconventional clinical features, including clinical, pathologic, and immunohistochemical features. Results: The first case developed in the left frontal lobe of a 20-year-old female with neurofibromatosis type 1 (NF1). Focal anaplastic features were present. The neoplastic cells were immunoreactive for GFAP, S-100 protein and focally for synaptophysin, with a MIB-1/Ki-67 proliferative labelling index of 16%. The second case developed in a 39-year-old female as a suprasellar neoplasm. The neoplastic cells expressed GFAP, S-100 protein and focally CD34. The adenohypophysis was positive for synaptophysin and pituicytes for TTF1. Molecular studies were negative for BRAF (V600E) mutation in both cases. Conclusion: PXA is a distinct circumscribed neoplasm that may present in unexpected locations or clinical backgrounds. Neuropathologists must be aware of these unconventional presentations in order to provide a precise diagnosis leading to appropriate treatment.

Original languageEnglish (US)
Pages (from-to)380-387
Number of pages8
JournalClinical neuropathology
Volume33
Issue number6
DOIs
StatePublished - 2014

Keywords

  • Anaplasia
  • BRAF
  • Neurofibromatosis
  • Pituitary
  • Pleomorphic xanthoastrocytoma
  • Sella

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology

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