Schwannomatosis

This code represents schwannomatosis, a rare genetic disorder characterized by the development of multiple schwannomas (benign tumors of the nerve sheath) throughout the body, excluding the vestibular nerves. Unlike neurofibromatosis type 2, patients with schwannomatosis typically do not develop bilateral vestibular schwannomas.

Use this code for patients diagnosed with schwannomatosis based on clinical criteria, genetic testing, or biopsy results confirming multiple non-vestibular schwannomas. Documentation should clearly indicate the diagnosis of schwannomatosis and differentiate it from other neurofibromatoses.

• Do not confuse this with neurofibromatosis type 1 (Q85.01) or neurofibromatosis type 2 (Q85.02).
• Ensure documentation specifies the type of neurofibromatosis or schwannomatosis to select the most accurate code.
• Code any associated manifestations or complications of schwannomatosis, such as pain or neurological deficits, in addition to this code.