Pleomorphic xanthoastrocytoma: Natural history and long-term follow-up

Cristiane M. Ida, Fausto J. Rodriguez, Peter C. Burger, Alissa A. Caron, Sarah M. Jenkins, Grant M. Spears, Dawn L. Aranguren, Daniel H. Lachance, Caterina Giannini

Research output: Contribution to journalReview articlepeer-review

116 Scopus citations


Prognostic significance of histological anaplasia and BRAF V600E mutation were retrospectively evaluated in 74 patients with pleomorphic xanthoastrocytoma (PXA). Median age at diagnosis was 21.5 years (31 pediatric, 43 adult) and median follow-up 7.6 years. Anaplasia (PXA-AF), defined as mitotic index-≥-5/10HPF and/or presence of necrosis, was present in 33 cases. BRAF V600E mutation was detected in 39 (of 60) cases by immunohistochemical and/or molecular analysis, all negative for IDH1 (R132H). Mitotic index-≥-5/10HPF and necrosis were associated with decreased overall survival (OS; P-=-0.0005 and P-=-0.0002, respectively). In all cases except two, necrosis was associated with mitotic index-≥-5/10HPF. Patients with BRAF V600E mutant tumors had significantly longer OS compared with those without BRAF V600E mutation (P-=-0.02). PXA-AF patients, regardless of age, had significantly shorter OS compared with those without (P-=-0.0003). Recurrence-free survival was significantly shorter for adult PXA-AF patients (P-=-0.047) only. Patients who either recurred or died ≤3 years from diagnosis were more likely to have had either PXA-AF at first diagnosis (P-=-0.008) or undergone a non-gross total resection procedure (P-=-0.004) as compared with patients who did not. This study provides further evidence that PXA-AF behaves more aggressively than PXA and may qualify for WHO grade III "anaplastic" designation.

Original languageEnglish (US)
Pages (from-to)575-586
Number of pages12
JournalBrain Pathology
Issue number5
StatePublished - Sep 1 2015


  • BRAF V600E
  • IDH1 R132H
  • WHO grade
  • anaplastic
  • glioma
  • pleomorphic xanthoastrocytoma

ASJC Scopus subject areas

  • General Neuroscience
  • Pathology and Forensic Medicine
  • Clinical Neurology


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