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Malignant Peripheral Nerve Sheath 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

Malignant peripheral nerve sheath tumors (MPNSTs) (previously called neurogenic sarcomas, malignant schwannomas, or neurofibrosarcomas) are soft tissue sarcomas, which arise from a peripheral nerve or show nerve sheath differentiation. MPNSTs are associated with a high risk of local recurrence and predominantly hematogenous metastasis. They account for 10 % of all soft tissue sarcomas, and approximately half of these malignancies arise in patients with neurofibromatosis type 1 (NF1). MPNSTs occur in about 2–5 % of patients with NF1 compared with an incidence of 0.001 % in the general population. In contrast, in a large population-based longitudinal study the lifetime risk of developing an MPNST in NF1 was 8–13 %. In patients with NF1, the majority of MPNSTs arise in a previous clinically detectable plexiform neurofibroma, but MPNST may also develop as a primary tumor.

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Authors and Affiliations

  1. Neuroblastoma Program, Division of Oncology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, MLC 7015, Cincinnati, OH, 45229, USA

    Brian Weiss M.D.

  2. Department of Pathology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA

    Amy Sheil M.D.

  3. Neural Tumors Program, Division of Experimental Hematology and Cancer Biology, Cincinnati Children’s Hospital, University of Cincinnati, Cincinnati, OH, USA

    Nancy Ratner Ph.D.

Authors
  1. Brian Weiss M.D.
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  2. Amy Sheil M.D.
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  3. Nancy Ratner Ph.D.
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Corresponding author

Correspondence to Brian Weiss M.D. .

Editor information

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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Weiss, B., Sheil, A., Ratner, N. (2015). Malignant Peripheral Nerve Sheath 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_16

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  • DOI: https://doi.org/10.1007/978-1-4939-1830-0_16

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  • Online ISBN: 978-1-4939-1830-0

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