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Neurocutaneous Syndromes

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Oncology of CNS Tumors

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Abstract

Neurocutaneous disorders are a heterogeneous group of genetic disorders characterized by abnormalities of the skin and nervous systems due to their common embryological origin. These disorders have inconsistent definitions, and therefore there is lack of consensus regarding which diseases belong to this category. In general, and for the purposes of this chapter, the neurofibromatoses, Sturge-Weber syndrome, von Hippel-Lindau syndrome, and Gorlin syndrome are accepted neurocutaneous disorders and will be discussed. Genetic aberrations in cell growth pathways are fundamental to all neurocutaneous syndromes which predispose these patients to developmental abnormalities and neoplasms of the central and peripheral nervous systems, skull, skin, and eyes. Therefore, these heterogeneous disorders are also known as tumor predisposition syndromes.

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Authors and Affiliations

  1. Division of Neurosurgery, The Ottawa Hospital, Ottawa, ON, Canada

    Michael S. Taccone

  2. Division of Neurosurgery, Department of Surgery, University of Ottawa, Ottawa, ON, Canada

    Michael S. Taccone

  3. Arthur & Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada

    Michael S. Taccone & James T. Rutka

  4. Division of Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada

    Michael S. Taccone & James T. Rutka

  5. Department of Surgery, University of Toronto, Toronto, ON, Canada

    James T. Rutka

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  1. Michael S. Taccone
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Correspondence to James T. Rutka .

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Editors and Affiliations

  1. Department of Neurosurgery, University of Munich Department of Neurosurgery, Munich, Germany

    Jörg-Christian Tonn

  2. Dana-Farber Cancer Institute Center, Harvard University, Boston, USA

    David A. Reardon

  3. SickKids Hospital, University of Toronto, Toronto, ON, Canada

    James T. Rutka

  4. Department of Neurosurgery, University of Hamburg, Hamburg, Germany

    Manfred Westphal

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Taccone, M.S., Rutka, J.T. (2019). Neurocutaneous Syndromes. In: Tonn, JC., Reardon, D., Rutka, J., Westphal, M. (eds) Oncology of CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-04152-6_19

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