• Jeffrey H Wisoff, Robert A Sanford, Linda A Heier, Richard Sposto, Peter C Burger, Allan J Yates, Emiko J Holmes, and Larry E Kun.
• Division of Pediatric Neurosurgery, NYU Langone Medical Center, New York, New York 10016, USA. jhw1@nyumc.org
• Neurosurgery. 2011 Jun 1; 68 (6): 1548-54; discussion 1554-5.

BackgroundCentral nervous system neoplasms are the most common solid tumors in children, and more than 40% are low-grade gliomas. Variable locations, extent of resection, postoperative neurodiagnostic evaluation, and histology have confounded therapy and outcome.ObjectivesTo investigate disease control and survival after surgery.MethodsA prospective natural history trial from 1991 to 1996 produced a subset of patients with low-grade gliomas managed by primary surgery and subsequent observation. Patients were evaluable if eligibility, tumor location, and extent of resection were confirmed by pathological diagnosis, preoperative and postoperative imaging, and the surgeon's report. Primary end points were overall survival (OS), progression-free survival (PFS), and postprogression survival.ResultsOf 726 patients enrolled, 518 were fully evaluable for analysis. The 5- and 8-year OS rates were 97% ± 0.8% and 96% ± 0.9%, respectively, and PFS rates were 80% ± 1.8% and 78% ± 2.0%. In univariate analyses, histological type, extent of residual tumor, and disease site were significantly associated with PFS and OS. In multivariate analysis, gross total resection (GTR) without residual disease was the predominant predictor of PFS. In patients with limited residual disease, 56% were free of progression at 5 years.ConclusionGTR should be the goal when it can be achieved with an acceptable functional outcome. The variable rate of progression after incomplete resection highlights the need for new predictors of tumor behavior.Copyright © 2011 by the Congress of Neurological Surgeons

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