What is Schwannomatosis?
Schwannomatosis is estimated to occur in 1 in 40,000 births. The inheritance pattern of Schwannomatosis is not well understood. The risk of transmitting the disorder to offspring is about 15%. Schwannomatosis symptoms usually first appear in adulthood.
Schwannomatosis is characterized by:
- Multiple schwannomas (nerve sheath tumors) without evidence of vestibular schwannoma
- Pain associated with the schwanomas
- Symptoms possibly limited to one area of the body
Diagnostic Criteria for Schwannomatosis
- Individual is age over 30 years
- AND has two or more non-intradermal Schwannomas, at least one with histological confirmation
- AND has no evidence of vestibular tumor on high-quality MRI scan
- AND has no known constitutional NF2 mutation/li>
Prognosis for Schwannomatosis
Anyone with schwannomatosis experiences some degree of pain, but the intensity varies. A small number of people have such mild pain that they are never diagnosed with the disorder. Most people have significant pain, which can be managed with medications or surgery. In some extreme cases, pain will be so severe and disabling it will keep people from working or leaving the house.
How is Schwannomatosis treated?
There is no currently accepted medical treatment or drug for schwannomatosis, but surgical management is often effective. Pain usually subsides when tumors are removed completely, although it may recur should new tumors form. When surgery isn’t possible, ongoing monitoring and management of pain in a multidisciplinary pain clinic is advisable.