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Pleomorphic xanthoastrocytoma in children and adolescents
Author(s) -
Rao Amulya A. Nageswara,
Laack Nadia N.,
Giannini Caterina,
Wetmore Cynthia
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22490
Subject(s) - medicine , pleomorphic xanthoastrocytoma , presentation (obstetrics) , adjuvant therapy , retrospective cohort study , surgery , pediatrics , cancer , glioma , astrocytoma , cancer research
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. Pediatr Blood Cancer. 2010;55:290–294. © 2010 Wiley–Liss, Inc.