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. (c) 2010 Wiley-Liss, Inc.
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. (c) 2010 Wiley-Liss, Inc.
Authors: Cristiane M Ida; Fausto J Rodriguez; Peter C Burger; Alissa A Caron; Sarah M Jenkins; Grant M Spears; Dawn L Aranguren; Daniel H Lachance; Caterina Giannini Journal: Brain Pathol Date: 2014-12-05 Impact factor: 6.508
Authors: Dora Dias-Santagata; Quynh Lam; Kathy Vernovsky; Natalie Vena; Jochen K Lennerz; Darrell R Borger; Tracy T Batchelor; Keith L Ligon; A John Iafrate; Azra H Ligon; David N Louis; Sandro Santagata Journal: PLoS One Date: 2011-03-29 Impact factor: 3.240