Schwannomatosis

ICD-10 Code Q85.03

Schwannomatosis

Definition

The least frequent form of the rare genetic disorder neurofibromatosis. It is clinically and genetically distinct from NF1 and NF2 and is characterized by the development of multiple schwannomas (nerve sheath tumors), without involvement of the vestibular nerves. NF3 develops in adulthood and is often associated with chronic pain. Dysesthesia and paresthesia may also be present. Common localizations include the spine, peripheral nerves, and the cranium.

Also known as neurilemmomatosis, Neurinomatosis, neurofibromatosis type 3, NF3, Schwannomatosis — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Nervous system Disease Has Primary Anatomic Site NCIT · CC BY 4.0