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Pleomorphic xanthoastrocytoma in children and adolescents
Amulya A Nageswara Rao1, Nadia N Laack, Caterina Giannini
1Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. nageswararao.amulya@mayo.edu
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.
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