Definition

What is a malignant peripheral nerve sheath tumor (MPNST)?

A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops in the protective lining that covers nerves.The first symptom of MPNST is often a lump or mass that increases in size, sometimes causing pain or a tingling sensation. MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.

How common is a malignant peripheral nerve sheath tumor (MPNST)?

Malignant peripheral nerve sheath tumors are rare. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of a malignant peripheral nerve sheath tumor (MPNST)?

The common symptoms of a malignant peripheral nerve sheath tumor (MPNST) are:

  • Pain in the affected area
  • Weakness when trying to move the affected body part
  • A growing lump of tissue under the skin

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes a malignant peripheral nerve sheath tumor (MPNST)?

It’s not clear what causes most malignant peripheral nerve sheath tumors.

Doctors know that these cancers begin when a cell in the protective lining around a nerve develops an error (mutation) in its DNA. The mutation tells the cell to multiply rapidly and to continue living when other cells would normally die. The accumulating cells form a tumor that can grow to invade nearby tissue or spread to other areas of the body.

Risk factors

What increases my risk for a malignant peripheral nerve sheath tumor (MPNST)?

There are many risk factors for a malignant peripheral nerve sheath tumor (MPNST), such as:

  • Previous radiation therapy for cancer. A malignant peripheral nerve sheath tumor may develop in the area treated with radiation 10 to 20 years after treatment.
  • Noncancerous nerve tumors. Malignant peripheral nerve sheath tumors can develop from noncancerous (benign) nerve tumors, such as neurofibroma.
  • An inherited condition that increases risk of nerve tumors. Malignant peripheral nerve sheath tumors occur more frequently in people with neurofibromatosis 1.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is a malignant peripheral nerve sheath tumor (MPNST) diagnosed?

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

  • Neurological examination. A detailed, comprehensive neurological examination helps your doctor understand your symptoms and gather clues about your diagnosis.
  • Imaging tests. Imaging tests help doctors understand the size of a tumor and look for signs that cancer has spread to other areas of the body. Imaging tests may include an MRI, magnetic resonance neurography, CT and positron emission tomography (PET).
  • Removing a sample of tissue for testing (biopsy). A biopsy sample may be obtained by a radiologist before surgery or by a surgeon during surgery. The tissue is sent to a laboratory for specialized testing. The information gathered in the lab may help your doctor better understand your prognosis and your treatment options.

How is a malignant peripheral nerve sheath tumor (MPNST) treated?

Treatment for malignant peripheral nerve sheath tumors often involves:

  • The goal of surgery is to remove the entire tumor and a small margin of healthy tissue that surrounds it. When that isn’t possible, surgeons remove as much of the tumor as they can. Depending on the location and size of your malignant peripheral nerve sheath tumor, surgery can cause nerve damage and disability. In the case of tumors that occur in the arms and legs, limb amputation may be necessary. In some cases your doctor may recommend radiation before surgery to shrink a tumor. That may make it more likely that the tumor can be removed completely.
  • Radiation therapy. Radiation therapy uses powerful beams of energy, such as X-rays and protons, to kill cancer cells. Radiation may be used before surgery to shrink a tumor and make successful surgery more likely. After surgery, radiation therapy may be used to kill any cancer cells that might remain.
  • Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. If your malignant peripheral nerve sheath tumor has spread to other parts of your body, your doctor may recommend chemotherapy to control your symptoms and slow the growth of the cancer.
  • After surgery, physical therapists and occupational therapists can help you recover function and mobility lost due to nerve damage or limb amputation.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage a malignant peripheral nerve sheath tumor (MPNST)?

Please discuss with your doctor for further information.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: November 22, 2017 | Last Modified: November 22, 2017

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