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Malignant peripheral nerve sheath tumor
Other Resources UpToDate PubMed

Malignant peripheral nerve sheath tumor

Contributors: Andrea Wasilewski MD, Christine Osborne MD, Jamie Adams MD, Benjamin L. Mazer MD, MBA, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed


Malignant peripheral nerve sheath tumors (MPNSTs), formerly known as neurofibrosarcomas, are tumors that grow around peripheral nerves. They account for less than 10% of peripheral nerve tumors. Patients present with a painful mass along a nerve distribution. Sensory and motor deficits of the nerve are common. MPNSTs tend to occur on the extremities and trunk and along major nerve trunks such as the brachial plexus.

Neurofibromas may transform into MPNSTs. This is of specific concern in patients with neurofibromatosis, who may have a high number of peripheral nerve tumors.

MPNSTs have a poor prognosis generally, with a worse prognosis in people with large tumors or neurofibromatosis. The primary treatment is surgical resection.


C47.9 – Malignant neoplasm of peripheral nerves and autonomic nervous system, unspecified

404037002 – Malignant peripheral nerve sheath tumor

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Solitary neurofibroma
  • Neurilemmoma
  • Perineurioma
  • Ganglioneuroma
  • Metastatic cancer
  • Neurolymphomatosis
  • Local hematoma with nerve compression
  • Cutaneous neuroma
  • Trauma (see Traumatic neuroma)
  • Vasculitis
  • Nerve infarction

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Last Reviewed:04/04/2019
Last Updated:04/22/2019
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Malignant peripheral nerve sheath tumor
A medical illustration showing key findings of Malignant peripheral nerve sheath tumor : Numbness, Paresthesias
Copyright © 2024 VisualDx®. All rights reserved.