Schwannomatosis is a new classification of disorders in the neurofibromatosis disorder. Neurofibromatosis affects the nerve cells and can cause tumors to grow on the nerves. It can also cause deformities of the skin and bones. A schwannoma is a benign (not cancerous) tumor that grows from beneath the nerve sheath (covering). Schwannomatosis is a disorder with many schwannomas that can appear on any nerve on the body except for the vestibular nerve, or the nerve that controls balance. The tumors are painless but as they grow, they can press on the nerves and cause pain.
In this article, the authors describe four patients with schwannomatosis, reviewing their symptoms and treatment. The first patient, patient 1, was a 31-year-old man who had a history of tumors on the nerve sheath. He came in with a progressively growing large mass on the left forearm and masses on both sides of the neck. The doctors also found that the patient had a tumor compressing his spinal cord, as well as a fww others in the arm that were only visible through magnetic resonance imaging (MRI). The second patient, patient 2, was a 37-year-old woman and patient 1’s sister. She had already had several surgeries to remove schwannomas from her arms and legs. This time, she came in because she was not able to walk after experiencing lower leg pain and weakness in both legs for the past month, and she had had loss of bladder control. The doctors found a large mass behind her right knee and one in her back that was pressing on the spinal nerves.
The third patient, patient 3, was a 44-year-old man who complained of increasing numbness, tingling, and pain in both hands and feet, and he had been unsteady on his feet for the past year. He also had a history of having tumors removed, as well as cancer of the rectum. Finally, the last patient, patient 4, was a 55-year-old man who complained of gradually increasing dizziness, hearing loss, and tinnitus (ringing in the ears). Thirty-five years earlier, he had had a schwannoma removed from his right cheek.
All four patients were treated with surgery, but patient 2 had to receive medication to reduce the swelling as well. All four patients recovered. Patient 1 did not have all the tumors removed and will have more treatment as they begin to cause symptoms. Patient 2 was able to walk again after surgery but was left with a bit of difficulty. The numbness and tingling remained in patient 3, and patient 4’s outcome wasn’t reported. The generally accepted treatment for schwannomas is surgery, but only if the tumors are causing problems.
The authors discussed the patients’ outcomes, stating that the removal of the tumors were successful and that none of the patients had worsened after the surgeries. After the schwannomas were tested, the patients were all diagnosed with schwannomatosis. This was important to note because the presence of multiple schwannomas does not immediately indicate a diagnosis of schwannomatosis.