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Journal of Child Neurology
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Using Neurofibromatosis-1 to Better Understand and Treat Pediatric Low-Grade Glioma

David H. Gutmann, MD, PhD

Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, gutmannd{at}wustl.edu

Relatively little is known about the seminal genetic events that trigger the development of low-grade gliomas in children. Genetically engineered mouse models of the neurofibromatosis-1—inherited tumor predisposition syndrome have identified key intracellular growth control pathways, defined the contribution of the tumor microenvironment to glioma growth, and helped researchers understand the genetic basis for glioma susceptibility. In addition, genetically engineered mouse low-grade glioma models have recently been used in preclinical therapeutic studies to evaluate the efficacy of particular biologically based therapies and to define outcome measures.

Key Words: Neurofibromin • astrocytoma • tumor microenvironment • preclinical therapeutics • brain tumor • genetically engineered mice

Journal of Child Neurology, Vol. 23, No. 10, 1186-1194 (2008)
DOI: 10.1177/0883073808321061
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