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Schwannomas

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Molecular Pathology of Nervous System Tumors

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

Abstract

Schwannomas are benign tumors of the cranial nerves and peripheral nervous system. They most commonly occur sporadically, but also in the context of a variety of tumor predisposition syndromes, including neurofibromatosis type 2 (NF2), schwannomatosis, and Carney’s complex. The majority of schwannomas are characterized by biallelic inactivation of the NF2 gene, resulting in the loss of expression of its gene product, the tumor suppressor Merlin. Other tumor suppressor genes implied in schwannoma biology include SMARCB1, PRKAR1A and most recently, LZTR1. Our increased understanding of the tumor genetics and molecular pathways driving schwannoma growth has led to the active development of molecular targeted therapies, some of which have already shown evidence of clinical activity in patients with schwannomas.

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Authors and Affiliations

  1. Division of Pediatric Hematology/Oncology, NYU Langone Medical Center, The Stephen D. Hassenfeld Children’s Center for Cancer and Blood Disorders, 160 East 32nd Street, 2nd Floor, New York, NY, 10016, USA

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

  2. Division of Neuropathology, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Warren Building, Rm 325, 55 Fruit St., Boston, MA, 02114, USA

    Anat Stemmer-Rachamimov M.D., F.R.C.P.(C).

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  1. Matthias A. Karajannis M.D., M.S.
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  2. Anat Stemmer-Rachamimov M.D., F.R.C.P.(C).
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Correspondence to Matthias A. Karajannis M.D., M.S. .

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Editors and Affiliations

  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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Karajannis, M.A., Stemmer-Rachamimov, A. (2015). Schwannomas. 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_15

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