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.