Schwannomatosis

From Wikipedia, the free encyclopedia

Schwannomatosis is one form of a genetic disorder called Neurofibromatosis (NF) that has only recently been recognized. It is a rare disorder, affecting only around 1:40,000 individuals.

[edit] Symptoms

• Schwannomas occur instead of Neurofibromas.
• Multiple Schwannomas occur.
• The Schwannomas develop on cranial, spinal and peripheral nerves.
• Chronic pain, and sometimes numbness, tingling and weakness.
• About 1/3 of patients have segmental Schwannomatosis, which means that the Schwannomas are limited to a single part of the body, such as an arm, a leg or the spine.
• Unlike the other forms of NF, the Schwannomas do not develop on vestibular nerves, and as a result, no loss of hearing is associated with Schwannomatosis.
• Patients with Schwannomatosis do not have learning disabilities related to the disease.

[edit] Cause

• Though known to be a genetic disorder, the gene for it has yet to be identified.
• Schwannomatosis can skip generations.

[edit] Treatment

• If feasible, the Schwannomas can be surgically removed. Any tumor-associated pain usually subsides after tumor removal.
• Sometimes, a tumor will reappear at the same site after surgery.
• If surgery is unfeasible, then pain management will have to be used.
• Other than surgery and/or pain management, there are no other medical treatments available. There are no drugs available to treat Schwannomatosis.
• It is important to note that Schwannomas are always benign, and never degenerate into cancer.