Definition and Diagnosis

These are cancerous lesions that arise from the connective tissue of peripheral nerves. They can occur spontaneously, in patients with neurofibromatosis, or following radiation therapy. They are similar to poorly differentiated sarcomas and can spread to the lungs via the blood stream. They usually present as a painful tumor that is growing rapidly. They often have a hard, immobile consistency, and may cause nerve irritation. MRI with and without contrast is important for delineating the tumor's extent, but definitive diagnosis requires a biopsy.

Treatment Options

Based on MRI appearance, if a total removal is possible without neurological deficit then surgery is recommended. If not, then an open biopsy is performed. If the tumor is malignant, obtaining clear margins is important, which may require the sacrifice of surrounding nerves and arteries, causing paralysis and numbness that is often irreversible. Re-operation may be recommended. Chemotherapy and regional radiation is often required to prevent recurrence.