Originally described in Japanese patients,[1] it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF.

[2] Schwannomas are mostly benign tumors that commonly occur in individuals with NF2 and schwannomatosis (sometimes called neurofibromatosis type III).

Myelin is a lipid covering that speeds the conduction of action potentials.

When Schwann cells proliferate out of control in an encapsulation it is called a schwannoma.

Schwannomas on sensory nerve axons cause chronic severe pain.

Treatment options for schwannomas are to surgically remove them, have radiation, cyberknife or intracapsular enucleation.

[4] An inactivating germline mutation in exon 1 of the tumor suppressor gene SMARCB1 has been reported in patients with schwannomatosis.