Pleomorphic xanthoastrocytoma in children and adolescents

Amulya A. Nageswara Rao, Nadia N Laack, Caterina Giannini, Cynthia Wetmore

Research output: Contribution to journalArticle

33 Scopus citations

Abstract

Background. Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor occurring primarily in children and young adults. The superficial location of the tumor facilitates gross total resection (GTR) thus conferring a relatively favorable outcome with a reported 10-year overall survival (OS) of 70%. Procedure. A retrospective case analysis of children and adolescents diagnosed and treated with PXA in our institution between January 1980 and March 2009 and a literature review. Results. 85.7% of our patients with a GTR were recurrence free. Only one of seven patients with less than a GTR did not recur and median time to recurrence was under 1 year in patients who did not have a GTR. Two of three patients with anaplastic features or malignant transformation at initial presentation progressed. Five-year OS and recurrence free survival (RFS) was 85.7% and 49%, respectively. Conclusions. GTR is the preferred treatment modality for PXA. Anaplastic features, though uncommon at initial presentation, confer a less favorable outcome. The role of adjuvant therapy with primary and recurrent anaplastic PXAs, especially when complete resection is not feasible, warrants further study.

Original languageEnglish (US)
Pages (from-to)290-294
Number of pages5
JournalPediatric Blood and Cancer
Volume55
Issue number2
DOIs
StatePublished - Aug 2010

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Keywords

  • Low-grade glioma
  • Management
  • Pleomorphic xanthoastrocytoma
  • Prognosis

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Pediatrics, Perinatology, and Child Health

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