Overview

Malignant peripheral nerve sheath tumors are the tumors that form in the lining of the nerves that extend from the spinal cord into the body. It can be found in any part of the body, especially the deep tissue of the arms, legs, and trunk. This rare type of cancer can cause the affected area to be painful and weak or have a lump that grows bigger over time.

Surgery is mostly recommended for malignant peripheral nerve sheath tumors, but radiation therapy and chemotherapy can also be alternatives in certain cases.

Symptoms

Signs and symptoms of malignant peripheral nerve sheath tumors include:

  • Constant pain
  • Weakness
  • Lump of tissue under skin

In case of persistent signs and symptoms, seeing a doctor is recommended.

Causes

Causes of malignant peripheral nerve sheath tumors are still unclear. All that is known for now is that the cancerous cells originate from genetic mutations happening in the protective lining around a nerve. This abnormality causes the affected cells to multiply out of control without dying, unlike normal cells. This leads to accumulations of abnormal cells which form a tumor that can spread to nearby tissue or any other parts of the body.

Risk factors

Contributing factors of malignant peripheral nerve sheath tumors include:

  • History of radiation therapy: to treat cancer, putting the radiated area at risk of formation of a malignant peripheral nerve sheath tumor in 10-20 years after treatment.
  • Noncancerous nerve tumors: such as neurofibroma, as they can become an original site of malignant peripheral nerve sheath tumors.
  • Inherited condition that increases risk of nerve tumors: such as Neurofibromatosis type 1.

Malignant peripheral nerve sheath tumors