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Neurofibromatosis Type 1 pp 341–352Cite as

NF1-Associated Optic Glioma

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Abstract

Gliomas are the most common central nervous system tumor encountered in individuals with NF1. These tumors are typically low-grade gliomas (pilocytic astrocytomas) involving the optic nerve, chiasm, and tracts (optic pathway gliomas), but they may also develop in the hypothalamus or brainstem of children with NF1. Over the past decade, several Nf1 genetically-engineered mouse models of optic glioma have been established. These instructive preclinical mouse strains have revealed new insights into the role of the NF1 protein (neurofibromin) in astrocyte and neural stem cell growth regulation as well as the importance of the tumor microenvironment and genomic modifiers to gliomagenesis and tumor maintenance. In this chapter, we review these new findings and discuss their implications for future therapeutic drug design.

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Acknowledgements

We thank the members of the Gutmann laboratory for their helpful comments during the preparation of this chapter. This work is partially supported by grants from the National Cancer Institute (U01-CA141549) and the James S. McDonnell Foundation.

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Authors and Affiliations

  1. Department of Neurology, Washington University School of Medicine, 8111, 660 South Euclid Avenue, St. Louis, MO, 63110, USA

    Anne C. Solga & David H. Gutmann

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  1. Anne C. Solga
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  2. David H. Gutmann
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Correspondence to David H. Gutmann .

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Editors and Affiliations

  1. School of Medicine, Institute of Medical Genetics, Cardiff University, Heath Park Campus, Cardiff, 144XN, United Kingdom

    Meena Upadhyaya

  2. School of Medicine, Institute of Medical Genetics, Cardiff University, Heath Park, Cardiff, CF14 4XN, United Kingdom

    David N. Cooper

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Solga, A.C., Gutmann, D.H. (2012). NF1-Associated Optic Glioma. In: Upadhyaya, M., Cooper, D. (eds) Neurofibromatosis Type 1. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-32864-0_22

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