Neurosurgery

Malignant Nerve Sheath Tumors

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About Malignant Nerve Sheath Tumors

General Information

  • Malignant nerve sheath tumors (MNST) occur along the sheaths that surround peripheral nerves. This classification includes tumors previously called malignant schwannoma, malignant neurofibroma, nerve sheath fibrosarcoma and neurofibrosarcoma.
  • MNSTs typically begin as a local growth. Metastasis, or transmission to other sites, occurs via the blood stream, most commonly to the lungs.
  • Approximately 40 percent to 60 percent of MNSTs arise as part of von Recklinghausen's disease, and 10 percent are generated by previous radiation treatment of tumors.
  • MNSTs account for 10 percent of soft-tissue sarcomas.

Symptoms

  • Pain is the key feature of an MNST.

Diagnosis

  • MNSTs typically involve a mass larger than 2 to 6 cm with irregular borders and a history of rapid expansion. MNSTs often produce neurological problems in the area served by the affected nerve.

Treatment

  • MNSTs are primarily treated by surgical removal. Surgery may require some loss of neurological function and radical excision of soft tissues.
  • The recurrence risk is high if the tumor is not removed completely. Post-operative radiation and chemotherapy are required.

Outcome

  • The reported five-year survival rate for a patient with a MNST is as high as 50 percent but drops to 10 percent in patients with von Recklinghausen's disease.