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Neurofibromatosis type 1 and associated malignancies

  • Kaleb YohayEmail author
Article

Abstract

Neurofibromatosis type 1 (NF1) is a common autosomal dominant neurocutaneous disorder with a predisposition to the development of benign and malignant tumors. Mutations in the NF1 gene result in loss of function of neurofibromin, a guanosine triphosphatase-activating protein that helps maintain the proto-oncogene Ras in its inactive form. Loss of neurofibromin results in increased proliferation and tumorigenesis. As a result, people with NF1 are at increased risk for the development of nervous and non-nervous system malignancies. Malignancy is a major source of morbidity and mortality in NF1. The natural history of NF1-associated malignancies is often different than that of their sporadic counterparts and, as such, management strategies need to be adjusted accordingly.

Keywords

Pilocytic Astrocytoma Malignant Peripheral Nerve Sheath Tumor Optic Pathway Optic Pathway Glioma Juvenile Myelomonocytic Leukemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Current Medicine Group, LLC 2009

Authors and Affiliations

  1. 1.Division of Child Neurology, Department of Pediatrics, Weill Cornell Medical CollegeNew York-Presbyterian HospitalNew YorkUSA

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