(from cft.org)

Features of Schwannomatosis

People with Schwannomatosis develop multiple Schwannomas on cranial, spinal and peripheral nerves–but they do not develop vestibular tumors and do not go deaf. They also do not develop any other kinds of tumors (for example, meningiomas, ependymomas or astrocytomas) and do not have learning disabilities.

People with Schwannomatosis have problems with chronic pain that often exceeds their neurological problems. The first symptom of Schwannomatosis is almost always pain, which can occur in any part of the body. Many patients with Schwannomatosis go several years before the source of their pain is realized because they have few or no neurological symptoms.

About Schwannomas

Both Schwannomas and neurofibromas originate in the insulating covering of peripheral nerves called the nerve sheath. Schwannomas are very homogenous tumors consisting ONLY of nerve sheath cells or Schwann cells. They stay on the outside of the nerve, but may push it aside or against a bony structure causing damage. Neurofibromas are very heterogeneous tumors, which incorporate all sorts of cells and structural elements in addition to the Schwann cells. They infiltrate the nerve and splay apart the individual nerve fibers. Schwanamatosis patients typically do not develop malignancies, although this occurs more frequently in NF1 and NF2.

Clinical Management

Surgical management of Schwannomatosis can be effective. When tumors are completely removed, pain often subsides — although it may recur if new tumors form. When surgery is not feasible, management in a multidisciplinary pain clinic is advisable.

There is no currently accepted medical treatment or drug for Schwannomatosis.