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Malignant Peripheral Nerve Sheath Tumors

Overview

Mayo Clinic is recognized internationally for its expertise in the treatment of malignant peripheral nerve sheath tumors (MPNSTs). Each year, Mayo Clinic surgeons perform more than 150 operations to remove both malignant (cancerous) and benign (noncancerous) peripheral nerve tumors, a large-volume practice for these relatively rare tumors.

At Mayo Clinic, experienced and highly trained specialists from neurology, neurosurgery, orthopedics, hematology, oncology, radiation oncology, physical medicine and rehabilitation and other specialties work together to diagnose malignant peripheral nerve sheath tumors and determine the most appropriate treatment for each patient. What might take months to accomplish in other settings may be done in days in Mayo's efficient multispecialty practice.

Mayo Clinic specialists also have extensive experience treating neurofibromatosis, a hereditary disorder characterized by tumors comprised of nervous system tissue and fibrous tissue. Nearly half of all cases of MPNSTs occur in patients with neurofibromatosis.

There is no known cure yet for MPNSTs, but Mayo's treatment approach has shown significant effectiveness in helping patients with MPNSTs maintain their body functions and enhance their quality of life. Mayo Clinic Cancer Center is a National Cancer Institute (NCI)-designated comprehensive cancer center. Patients at Mayo Clinic are assured access to new and experimental treatments for MPNST as they develop.

Mayo Clinic in Rochester, Minnesota, is ranked No. 1 in Neurology & Neurosurgery in the U.S. News & World Report Best Hospitals rankings.

Diagnosis

MPNSTs can be fast-growing tumors. Accurate, detailed scans are critically important. Mayo Clinic provides many tools to help specialists clarify abnormal findings and ensure an accurate diagnosis. Mayo specialists may use magnetic resonance imaging (MRI) or positron emission tomography (PET) scans to provide detailed pictures of malignant peripheral nerve sheath tumors and computerized tomography (CT) to screen for other tumors.

Read more about malignant peripheral nerve sheath tumor diagnosis at Mayo Clinic.

Treatment

Treatment of malignant peripheral nerve sheath tumors can help extend the patient's length and quality of life.

At Mayo Clinic, wide resection (complete removal) of the tumor, when possible, often is performed in combination with radiation and chemotherapy. Chemotherapy and radiation treatments may be recommended preoperatively to shrink tumors before they are surgically removed. In some cases, Mayo Clinic specialists may administer radiation during surgery and postoperatively as well.

Read more about malignant peripheral sheath tumor treatment options at Mayo Clinic.

About Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumors form on the peripheral nerves (nerves that travel out from the brain and spinal cord, stimulating the muscles). The nerve sheath is the soft tissue surrounding the nerve.

Malignant peripheral nerve sheath tumors are a type of soft tissue sarcoma. They occur most commonly in deep soft tissue near the sciatic nerve, brachial plexus and sarcal plexus.

MPNSTs are the cancerous version of noncancerous soft tissue tumors such as neurofibromas and schwannomas. These cancerous tumors can occur in many parts of the body, such as the legs or arms, or in the abdomen, chest or pelvic areas of the body.

Five percent to 10 percent of individuals affected by neurofibromatosis, an inherited condition that causes peripheral nerve tumors, develop malignant peripheral nerve sheath tumors.

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