Schwannomatosis is a rare form of NF that has only recently been recognized. In this condition, multiple schwannomas may develop in the body, and can be very painful. Most often it affects the cranial, spinal, and peripheral nerves. Unlike neurofibromatosis, type 2, it does not affect the auditory nerves. Features vary greatly between people who have this condition. It appears to affect around 1 in 40,000 individuals.
Schwannomatosis is a genetic condition, but it does not follow a clear pattern of inheritance. Researchers are still trying to understand the specific gene(s) involved.
Because schwannomatosis has so recently been identified, there are no official diagnostic criteria. Please refer to the following website for ongoing updates: http://www.ctf.org/.
There is no currently accepted medical treatment or drug for schwannomatosis. Surgical management of schwannomatosis can be effective. When tumors are completely removed, pain often subsides - although it may recur if new tumors form. When surgery is not feasible, management in a multidisciplinary pain clinic is advisable.