Diagnosis

Tests and procedures used to diagnose malignant peripheral nerve sheath tumors include:

  • Neurological exam. A detailed exam of the nervous system, known as a neurological exam, helps a health care provider gather clues for diagnosis.
  • Imaging tests. Imaging tests make pictures of the body. The pictures might help providers see the size of the cancer and whether it has spread to other parts of the body. Tests might include magnetic resonance imaging, also called MRI, or magnetic resonance neurography. Other tests might include computed tomography, also called CT scan, and positron emission tomography, also called PET scan.
  • Removing a sample of tissue for testing. A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample.

    The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. The health care team uses this information to make a treatment plan.

Treatment

Treatment for malignant peripheral nerve sheath tumors often involves:

  • Surgery. The goal of surgery is to remove the tumor and some of the healthy tissue around it. When that can't be done, surgeons remove as much of the tumor as they can.

    Depending on where a malignant peripheral nerve sheath tumor is and how big it is, surgery can cause nerve damage. In the case of tumors that occur in the arms and legs, surgically removing the arm or leg might be necessary.

    Sometimes, radiation before surgery might shrink a tumor. That might make it more likely that all of the tumor is removed during surgery.

  • Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

    Radiation may be used before surgery to shrink a tumor. This might make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill any cancer cells that might remain.

  • Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells. Chemotherapy might be an option when a malignant peripheral nerve sheath tumor has spread to other parts of the body. Chemotherapy might help control symptoms and slow the growth of the cancer.
  • Rehabilitation. After surgery, physical therapists and occupational therapists can help you regain function and movement lost due to nerve damage or from removing an arm or leg.

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Preparing for your appointment

If you have symptoms that worry you, make an appointment with your doctor.

If you're diagnosed with a malignant peripheral nerve sheath tumor, you'll likely be referred to a doctor who specializes in:

  • Conditions that affect the nervous system, known as a neurologist.
  • Treating cancer, known as an oncologist.
  • Surgery involving bones, known as an orthopedist.
  • Surgery involving nerves, known as a neurosurgeon.

Because appointments can be brief and because there's a lot to discuss, it's a good idea to be prepared. Here's some information to help you get ready and know what to expect.

What you can do

Ask a family member or friend to go with you. Sometimes it can be hard to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.

Make a list of:

  • Your symptoms, and when they began.
  • All medicines, vitamins and other supplements you take, including doses.
  • Questions to ask your health care provider.

Some questions to ask at your first appointment include:

  • What may be causing my symptoms or condition?
  • Are there other possible causes?
  • What tests do I need?
  • What's the next step in finding my diagnosis and deciding on treatment?
  • Are there restrictions that I need to follow in the meantime?

Questions to ask of a specialist:

  • Do I have a malignant peripheral nerve sheath tumor?
  • What are the goals of treatment for me?
  • What treatment do you suggest?
  • Do I need to begin treatment right away?
  • I have these other health problems. How can I best manage them together?
  • What are the possible side effects of treatment?
  • If the treatment doesn't work, what's next?
  • What is the outlook for my condition?

Be sure to ask other questions you have.

What to expect from your doctor

Your health care provider is likely to ask you questions, including:

  • Have your symptoms changed over time?
  • Does anything make your symptoms better?
  • Does anything make them worse?
  • Have you been told you have other medical conditions?
July 31, 2024

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  1. Gilchrist JM, et al. Peripheral nerve tumors. https://www.uptodate.com/contents/search. Accessed Jan. 23, 2023.
  2. Winn HR, ed. Benign and malignant tumors of the peripheral nerve. In: Youmans and Winn Neurological Surgery. 8th ed. Elsevier; 2023. https://www.clinicalkey.com. Accessed Jan. 23, 2023.
  3. Malignant peripheral nerve sheath tumor. National Cancer Institute. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/mpnst. Accessed Jan. 23, 2023.
  4. Nimmagadda R. Allscripts EPSi. Mayo Clinic. May 5, 2022.
  5. Cai Z, et al. Prognosis and risk factors for malignant peripheral nerve sheath tumor: A systematic review and meta-analysis. World Journal of Surgical Oncology. 2020; doi:10.1186/s12957-020-02036-x.

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