Pleomorphic xanthoastrocytoma in children and adolescents.
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%.


METHODS
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.
Rao
Amulya A Nageswara
AA
Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. nageswararao.amulya@mayo.edu
Laack
Nadia N
NN
Giannini
Caterina
C
Wetmore
Cynthia
C
D000293:Adolescent; D001254:Astrocytoma / Q000401:mortality / Q000473:pathology / Q000628:therapy; D001932:Brain Neoplasms; D002648:Child; D003131:Combined Modality Therapy; D003952:Diagnostic Imaging; D006801:Humans; D019635:Neurosurgical Procedures / Q000706:statistics & numerical data; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome