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Hereditary Predisposition to Primary CNS Tumors

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Molecular Pathology of Nervous System Tumors

Part of the book series: Molecular Pathology Library ((MPLB,volume 8))

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Abstract

The majority of primary central nervous system (CNS) tumors in children and adults occur sporadically, i.e., in patients with an unremarkable family history and no clinical evidence of a hereditary disorder, such as a tumor predisposition syndrome. Nevertheless, some brain tumors arise in the setting of such syndromes, such as neurofibromatosis, and the underlying condition is often not recognized until after a tumor is diagnosed. Tumors arising in patients with such syndromes sometimes have a different biological behavior compared to their sporadic counterparts, afflicted patients may require additional treatments or screening, and multiple family members may be affected. Therefore, it is important for those involved in the diagnosis, treatment or follow-up of brain tumor patients to be knowledgeable about known hereditary predispositions relevant to this population. As genome-wide sequencing is increasingly applied, it can be expected that additional hereditary predispositions to CNS tumors will be identified in the near future.

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Authors and Affiliations

  1. Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Uri Tabori M.D.

  2. Department of Haematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada

    Uri Tabori M.D.

  3. Division of Pediatric Hematology/Oncology, NYU Langone Medical Center, 160 East 32nd Street, 2nd Floor, New York, NY, 10016, USA

    Matthias A. Karajannis M.D., M.S.

  4. Division of Clinical Genetic Services, Department of Pediatrics, NYU Langone Medical Center, 145 East 32nd St., 14th Fl, New York, NY, 10016, USA

    John G. Pappas M.D., M.S.

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  1. Uri Tabori M.D.
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Correspondence to Matthias A. Karajannis M.D., M.S. .

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  1. Associate Professor of Pediatrics and Otolaryngology, NYU Langone Medical Center, New York, New York, USA

    Matthias A. Karajannis

  2. Professor of Pathology and Neurosurgery, NYU Langone Medical Center, New York, New York, USA

    David Zagzag

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Tabori, U., Karajannis, M.A., Pappas, J.G. (2015). Hereditary Predisposition to Primary CNS Tumors. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_1

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