Definition and Diagnosis

MRI of the right brachial plexus revealing two schwannomas (asterisks). This patient schwannomatosis had a previous schwannoma removed from a thoracic spinal nerve, and has no evidence of eighth cranial nerve tumors.

This disease is characterized by schwannomas on multiple nerves throughout the body, usually in the spine, neck, and limbs. Because bilateral eight cranial nerve tumors do not occur, this disease can be differentiated from neurofibromatosis type 2. Although patients with this condition often live normal lives, chronic pain may become a problem in a small percentage of those affected. As with neurofibromatosis, the severity of schwannomatosis varies greatly between patients. A diagnosis is made when multiple schwannomas occur without evidence of eight cranial nerve tumors.

Treatment Options

Tumors are removed when they become symptomatic (e.g., pain, weakness, or numbness). Patients are educated to monitor themselves for early signs of any new tumors, so that surgery, if required, can be performed when the tumors are small and easy to remove. With close medical care, patients do very well.