Medical Centric

MALIGNANT PERIPHERAL NERVE SHEATH TUMOR

Men's Health, Women's Health / By

MALIGNANT PERIPHERAL NERVE SHEATH TUMOR

  • A malignant peripheral nerve sheath tumor (MPNST) is a tumor that occurs in the lining of the nerves that extend from the spinal cord into the body.
  • Malignant peripheral nerve sheath tumors used to be 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.
  • They tend to cause pain and weakness in the affected area and may also cause a growing lump or mass.

SYMPTOMS

Symptoms of sarcoma can vary depending on the size and location of your tumor.You may experience all, some, or none of these symptoms before you are diagnosed with a sarcoma:

  • A lump or swelling in the in the soft tissue of the body under the skin, often on the arm, leg or trunk
  • increasing in size
  • is larger than 5cm
  • usually painful, but not always

CAUSES

  • 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.

DIAGNOSIS AND TREATMENT

Some diagnosis that a doctor may carry out are:

  • Neurological examination.
  • Removing a sample of tissue for testing (biopsy).
  • Imaging tests.

Successful treatments include:

  • Chemotherapy
  • Surgery
  • Radiation therapy
  • Rehabilitation 

RISK FACTORS

  • Factors that increase the risk of malignant peripheral nerve sheath tumors include:
  • 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.