• Erik J. Uhlmann
  • Scott R. PlotkinEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 724)


The studies of familial tumor predisposition syndromes have contributed immensely to our understanding of oncogenesis. Neurofibromatosis 1, neurofibromatosis 2 and schwannomatosis are inherited autosomal dominant neurocutaneous disorders with complete penetrance. They are clinically and genetically distinct and considerable knowledge has been gathered about their pathogenesis. In this chapter, the genetics, molecular mechanism of disease, as well as clinical features, diagnosis and treatment are discussed.


Neurodegenerative Disease Malignant Peripheral Nerve Sheath Tumor Intracranial Meningioma Hippo Signaling Tumor Suppressor Pathway 
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

© Landes Bioscience and Springer Science+Business Media 2012

Authors and Affiliations

  1. 1.Stephen E. and Catherine Pappas Center for Neuro-OncologyMassachusetts General HospitalBostonUSA

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