Primary meningeal pleomorphic xanthoastrocytoma with anaplastic features: A report of 2 cases, one with BRAF<sup>V600E</sup> mutation and clinical response to the BRAF inhibitor dabrafenib

Aisulu Usubalieva, Christopher R. Pierson, Christina A. Kavran, Kristin Huntoon, Oleksandr Kryvenko, Theodore G. Mayer, Weiqiang Zhao, Jack Rock, Mario Ammirati, Vinay K. Puduvalli, Norman L. Lehman

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

Primary meningeal gliomas are rare tumors composed of a heterogeneous group of neoplasms. We present 2 clinically aggressive cases of primary meningeal pleomorphic xanthoastrocytoma that clinically mimicked meningioma. One case presented in the posterior fossa of a 56-year-old woman; the other centered on the left operculum of a 35-year-old woman. These cases showed many of the classic features of pleomorphic xanthoastrocytoma, except that xanthomatous cells were rare and eosinophilic granular bodies were inconspicuous. Both cases exhibited high proliferative indices and superficially invaded the brain. One case harboring a BRAF<sup>V600E</sup> mutation disseminated to the thecal sac and showed a clinical response to the targeted BRAF inhibitor dabrafenib. These cases seem to represent an unusual primarily extra-axial presentation of pleomorphic xanthoastrocytoma and may account for at least some of the previously reported cases of primary meningeal glioma and/or glial fibrillary acidic protein-immunoreactive meningioma variants. We suggest that BRAF mutation analysis be considered in all meningeal lesions showing atypical histologic or immunohistochemical profiles, particularly those exhibiting glial differentiation, as a diagnostic aid and possible indication for targeted therapy.

Original languageEnglish (US)
Pages (from-to)960-969
Number of pages10
JournalJournal of Neuropathology and Experimental Neurology
Volume74
Issue number10
StatePublished - Oct 1 2015

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Keywords

  • Anaplastic features
  • BRAF<sup>V600E</sup>
  • Extra-axial
  • High mitotic rate
  • High proliferation index
  • Meningeal
  • Pleomorphic xanthoastrocytoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Neurology
  • Cellular and Molecular Neuroscience

Cite this

Usubalieva, A., Pierson, C. R., Kavran, C. A., Huntoon, K., Kryvenko, O., Mayer, T. G., Zhao, W., Rock, J., Ammirati, M., Puduvalli, V. K., & Lehman, N. L. (2015). Primary meningeal pleomorphic xanthoastrocytoma with anaplastic features: A report of 2 cases, one with BRAF<sup>V600E</sup> mutation and clinical response to the BRAF inhibitor dabrafenib. Journal of Neuropathology and Experimental Neurology, 74(10), 960-969.