Your peripheral nervous system includes nerves that travel out from your brain and spinal cord (your central nervous system) to other parts of your body. The nerve sheath is the soft tissue that covers the nerve. A malignant peripheral nerve sheath tumor (MPNST) is cancerous — a soft tissue sarcoma that forms from peripheral nerve sheath cells or from noncancerous (benign) peripheral nerve sheath tumors called neurofibromas.

MPNSTs occur most commonly in deep soft tissue along the nerves that run from the buttocks to legs (sciatic nerves), neck to arms or within the pelvis, but they can occur elsewhere. They can be fast growing, with few or no symptoms in the earliest stages, and cause pain or nerve loss as the tumors grow. A small percentage of people who have neurofibromatosis, an inherited condition that causes neurofibromas, develop MPNSTs.

  • Experience and expertise. Mayo Clinic is nationally recognized for experience and expertise in peripheral nerve surgery and neurofibromatosis, and is one of the leading centers for diagnosing and treating peripheral nerve tumors.
  • Teamwork. At Mayo Clinic, a team of experienced and highly trained specialists from neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to diagnose MPNSTs and determine the most appropriate treatment for you. Mayo's multispecialty treatment approach effectively helps people with MPNSTs to maintain function and improve quality of life.
  • Latest treatments. While there is no known cure for MPNSTs, best results are usually achieved with surgery, often combining radiation or chemotherapy. At Mayo Clinic you have access to new and experimental treatments for MPNSTs.
  • Comprehensive cancer center. Mayo Clinic Cancer Center meets strict standards for a National Cancer Institute comprehensive cancer center, recognizing scientific excellence and a multispecialty approach focused on cancer prevention, diagnosis and treatment.

Mayo Clinic in Rochester, Minn., and Mayo Clinic in Scottsdale, Ariz., are ranked among the Best Hospitals for cancer by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., is ranked high performing for cancer by U.S. News & World Report.

At Mayo Clinic, we assemble a team of specialists who take the time to listen and thoroughly understand your health issues and concerns. We tailor the care you receive to your personal health care needs. You can trust our specialists to collaborate and offer you the best possible outcomes, safety and service.

Mayo Clinic is a not-for-profit medical institution that reinvests all earnings into improving medical practice, research and education. We're constantly involved in innovation and medical research, finding solutions to improve your care and quality of life. Your doctor or someone on your medical team is likely involved in research related to your condition.

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Mayo Clinic's team of experienced specialists uses the results of a thorough physical and neurological exam and a complete medical history, along with critically important detailed scans, to provide accurate diagnosis of malignant peripheral nerve sheath tumors (MPNSTs). In tests that involve radiation, specialists carefully monitor doses to avoid the risk of radiation overexposure. Tests may include:

Read more about MRI, CT scan, PET scan and biopsy.

Depending on the size and location of the MPNST, treatment may include:

  • Surgery. The main treatment is to remove as much of the cancerous tumor as possible (surgical resection), ideally with no cancer detected at the edges of the removed tissue (negative margins). Mayo surgeons try to minimize damage to nearby muscle and nerve tissue to the extent possible.
  • Radiation therapy and chemotherapy. In cases where surgery cannot remove all of the cancer, specialists may recommend radiation therapy before, during or after surgery. Your doctor may recommend intensity-modulated radiation therapy (IMRT) or 3-D conformal radiation therapy. In some cases, chemotherapy also may be needed.
  • Rehabilitation. After surgery, the muscles controlled by the nerve from which the cancerous tumor was removed may need to be positioned or exercised to keep the affected muscles and joints active, prevent stiffness, and help restore function and feeling. Experienced physical and occupational therapists will guide you through specific exercises and provide braces or splints if necessary. Your doctor may recommend medication for pain control.

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 in Arizona, experienced and highly trained specialists from neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to diagnose and treat adults with malignant peripheral nerve sheath tumors (MPNSTs).

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 in Florida, experienced and highly trained specialists from neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to diagnose and treat adults and children with MPNSTs.

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 in Minnesota, experienced and highly trained specialists from neurology, neurosurgery, orthopedic surgery, oncology, radiation oncology, physical medicine and rehabilitation, genetic counseling, and other specialties work together to diagnose and treat adults and children with MPNSTs.

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.

Children with MPNSTs are treated at Mayo Clinic in Minnesota and Florida.

In Minnesota, highly skilled pediatric oncologists use the latest advances in treatments to diagnose and treat MPNSTs. Children who need inpatient care stay at Mayo Eugenio Litta Children's Hospital.

In Florida, pediatric oncologists from Nemours Children's Clinic and skilled surgeons from Mayo Clinic work as a team to provide the most appropriate care for each child.

Mayo Clinic is a member of the Children's Oncology Group. This national consortium of cancer centers establishes effective treatment approaches for treating pediatric soft tissue sarcomas, including MPNSTs.

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.

Specialists at Mayo Clinic are among the nation's leading cancer researchers and develop protocols that become standard procedures. To provide the most appropriate treatment for malignant peripheral nerve tumors (MPNSTs), Mayo Clinic participates in national clinical trials of new treatment approaches, such as:

  • Combinations of different types of chemotherapy, radiation therapy and surgery
  • Nerve surgery
  • The mechanisms of MPNSTs
  • Nerve regeneration and grafting

Publications

See a list of publications written by Mayo Clinic doctors about MPNSTs at PubMed, a service of the National Library of Medicine.

Jun. 27, 2013