Schwannomatosis Fact Sheet


Schwannomatosis is the most recently recognized form of neurofibromatosis.
It occurs in approximately 1 in 40,000 births, and is therefore the rarest form of NF.

The onset of symptoms is usually at puberty or older for both males and females. The precise genetic mechanism of Schwannomatosis is unknown at this time.

The identifying feature of Schwannomatosis is the growth of multiple schwannomas everywhere in the body but on the vestibular nerve.

The dominant symptom is pain, which comes about as a schwannoma enlarges, compresses nerves, or presses on nearby tissue.

Some people experience additional neurological symptoms like numbness, tingling, or weakness in the extremities.

Individuals with Schwannomatosis do not have neurofibromas.

About one-third of individuals with Schwannomatosis have tumors limited to a single part of the body, such as an arm, leg, or a part of the spine.

Some people develop many schwannomas, while others develop only a few.