page_title: Malignant peripheral nerve sheath tumor
section_title: Signs and symptoms
hierarchical_section_title: Malignant peripheral nerve sheath tumor / Signs and symptoms
caption_attribution_description: English: MRI image showing the site of the tumor in the left tibia
context_page_description: A malignant peripheral nerve sheath tumor is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.
  The first-line treatment is surgical resection with wide margins. Chemotherapy and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.
context_section_description: Symptoms may include:
  Swelling in the extremities (arms or legs), also called peripheral edema; the swelling often is painless.
  Difficulty in moving the extremity that has the tumor, including a limp.
  Soreness localized to the area of the tumor or in the extremity.
  Neurological symptoms.
  Pain or discomfort: numbness, burning, or "pins and needles."
  Dizziness and/or loss of balance.
caption: MPNST
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