Pleomorphic xanthoastrocytoma in children and adolescents

Pediatr Blood Cancer. 2010 Aug;55(2):290-4. doi: 10.1002/pbc.22490.


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.

MeSH terms

  • Adolescent
  • Astrocytoma* / mortality
  • Astrocytoma* / pathology
  • Astrocytoma* / therapy
  • Brain Neoplasms
  • Child
  • Combined Modality Therapy
  • Diagnostic Imaging
  • Humans
  • Neurosurgical Procedures / statistics & numerical data
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome