Malignant peripheral nerve sheath tumors are a type of cancer that occurs in the protective lining of the nerves that extend from the spinal cord into the body. Malignant peripheral nerve sheath tumors are also called neurofibrosarcomas.

Malignant peripheral nerve sheath tumors can occur anywhere in the body, but most often occur in the deep tissue of the arms, legs and trunk. Malignant peripheral nerve sheath tumors tend to cause pain and weakness in the affected area and may also cause a growing lump or mass.

Malignant peripheral nerve sheath tumors occur more frequently in people with an inherited condition that causes nerve tumors (neurofibromatosis) and in people who have undergone radiation therapy for cancer. However, most people with malignant peripheral nerve sheath tumors have no risk factors for the disease.

Malignant peripheral nerve sheath tumors are typically treated with surgery. In certain cases, radiation therapy and chemotherapy also may be recommended.

  • Collaboration. At Mayo Clinic, neurologists, neurosurgeons, orthopedic surgeons, oncologists, radiation oncologists, pathologists and radiologists, physical medicine and rehabilitation specialists, and genetic counselors work as a multidisciplinary team to provide whole-person care for people with malignant peripheral nerve sheath tumors.
  • Experience. Mayo Clinic doctors have extensive experience caring for people with malignant peripheral nerve sheath tumors. Your care team is prepared with the knowledge and resources to provide you with exactly the care you need.
  • Expertise. Surgery to remove malignant peripheral nerve sheath tumors requires the skills of a highly trained surgeon. Mayo Clinic doctors are recognized for their expertise in this area.
  • Access to the latest. Mayo Clinic doctors have access to the latest diagnostic and treatment options for malignant peripheral nerve sheath tumors.
  • A full range of treatment options to consider. Mayo Clinic doctors will work with you to review all of your treatment options and choose the treatment that best suits your needs and goals.
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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 MRI, magnetic resonance neurography, CT and positron emission tomography (PET).
  • Removing a sample of tissue for testing (biopsy). A biopsy may be obtained by a radiologist before surgery or by a surgeon during surgery. Using sophisticated laboratory processes, a doctor who specializes in analyzing blood and body tissue (pathologist) analyzes the tumor cells. The information gathered in the lab may help your doctor better understand your prognosis and your treatment options.

Treatment for malignant peripheral nerve sheath tumors often involves:

  • Surgery. 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.

    Mayo Clinic surgeons are highly trained and experienced at removing nerve tumors. Surgeons work to maximize function, while removing as much of the cancer as possible.

  • Radiation therapy. Radiation therapy uses powerful beams of energy 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.

    Mayo Clinic radiation oncologists create precise treatment plans that use advanced technology to carefully focus radiation and minimize harm to nearby healthy tissue.

  • Chemotherapy. 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 the body, your doctor may recommend chemotherapy to control your symptoms and slow the growth of the cancer.
  • Rehabilitation. After surgery, experienced physical and occupational therapists can help you recover function and mobility lost due to nerve damage or limb amputation.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

At Mayo Clinic, experienced and highly trained experts in neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to provide whole-person care for people with malignant peripheral nerve sheath tumors.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic, experienced and highly trained experts in neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to provide whole-person care for people with malignant peripheral nerve sheath tumors.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic, experienced and highly trained experts in neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, medical genetics, and other specialties work together to provide whole-person care for people with malignant peripheral nerve sheath tumors.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Mayo Clinic researchers are studying new ways to diagnose and treat malignant peripheral nerve sheath tumors.

Cancer research is conducted in coordination with the Mayo Clinic Cancer Center. The Mayo Clinic Cancer Center receives funding from the National Cancer Institute and is designated as a comprehensive cancer center — recognition for an institution's scientific excellence and multidisciplinary resources focused on cancer prevention, diagnosis and treatment.

See a list of publications by Mayo Clinic authors on malignant peripheral nerve sheath tumors on PubMed, a service of the National Library of Medicine.

Sep. 03, 2014