Schwannomatosis is the rarest type of Neurofibromatosis, as it only is seen in approximately 1 in 40,000 individuals. Diagnosis of Schwannomatosis is generally very difficult, and it has only been in recent years that Schwannomatosis has been recognized as a different subtype of neurofibromatosis.
Schwannomatosis does not have a clear pattern of inheritance like NF-1 and NF-2 do, and individuals tend to not show symptoms of the disorder until they are in their forties.
Diagnostic criteria for Schwannomatosis remains vague, and the first symptom that usually
presents itself is pain. Pain can go undiagnosed for several years because these patients almost never
present with other neurological problems or deficits.
People with this condition do have multiple Schwannomas on different nerves, but they are
never seen on the vestibulocochlear nerve like in NF-2. These kinds of tumors tend to stay on the
outside of nerves, but they may push it aside or against a bony structure causing damage. This damage
may be irreversible, but removal of these tumors often provides a great deal of pain relief.
There are no clinically accepted drugs for the treatment of Schwannomatosis, so surgery is the
primary method of treatment.