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Peripheral Nerve Sheath Tumors

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Oncology of CNS Tumors

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Abstract

Peripheral nerve sheath tumors (PNST) are a relatively uncommon soft tissue neoplasm that can arise from anywhere on the body and range from benign tumors, such as schwannomas and cutaneous neurofibromas, to high-grade malignant tumors referred to as malignant peripheral nerve sheath tumors (MPNST). There are major challenges in the diagnosis and management of PNSTs, which require a multidisciplinary team of surgeons, radiologists, neuropathologists, and medical oncologists with expertise in PNSTs at specialized centers. Magnetic resonance imaging (MRI) is the best imaging option to identify the presence of a PNST, although it cannot reliably distinguish between the subtypes of benign PNSTs (schwannoma vs. neurofibroma) or distinguish between benign PNST and MPNST. Surgery is not always indicated for benign PNST, especially when the patient is asymptomatic and the tumor can be followed with serial imaging. However, when a tumor becomes symptomatic with pain or neurological symptoms or has clinical suspicion for malignant transformation, surgery is warranted. The goals of surgery can vary from gross total resection to subtotal resection to biopsy based on the histopathological subtype of the PNST. In this chapter, we provide a review on the diagnosis, management, and outcomes of PNST.

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Authors and Affiliations

  1. MacFeeters-Hamilton Centre for Neuro-Oncology Research, Toronto Medical Discovery Tower, University of Toronto, Toronto, ON, Canada

    Suganth Suppiah, Shirin Karimi & Gelareh Zadeh

Authors
  1. Suganth Suppiah
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  2. Shirin Karimi
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  3. Gelareh Zadeh
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Corresponding author

Correspondence to Gelareh Zadeh .

Editor information

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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Suppiah, S., Karimi, S., Zadeh, G. (2019). Peripheral Nerve Sheath Tumors. 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_38

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  • DOI: https://doi.org/10.1007/978-3-030-04152-6_38

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-04151-9

  • Online ISBN: 978-3-030-04152-6

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