Neurofibromas are benign tumors of peripheral nerves. They arise from the cells that form and support the nerve sheath—Schwann cells, fibroblasts, and perineural cells. These tumors infiltrate the nerve and disrupt the sheaths of individual fibers. The most commonly affected nerve is the vestibulocochlear nerve, which transmits sound and balance information to the brain from the inner ear. Neurofibromas can be single or multiple. When multiple, they are associated with neurofibromatosis type I, a genetic disorder also known as von Recklinghausen disease.
Symptoms depend on the location and size of the tumor. These tumors typically are a painless, slow-growing mass, and may cause no symptoms.
The preferred surgical treatment at UPMC for neurofibroma is the Endoscopic Endonasal Approach (EEA). This innovative technique involves using the nose and nasal cavities to remove the tumors. EEA offers the benefits of no incisions to heal, no disfigurement to the patient, and a shorter recovery time.
Your doctor will perform a physical exam and ask about any symptoms you are experiencing. Symptoms depend on the location and size of the tumor. These tumors typically are a painless, slow-growing mass. You may feel an electric-like shock when light pressure is applied to the tumor. If a motor or sensory nerve is involved, the associated function may be affected.
Neurofibromas have a characteristic appearance on MRI scans. A biopsy of the tumor taken during surgery enables positive diagnosis.
Surgery is the treatment of choice for neurofibromas; however, it is complicated by the fact that these tumors are often interwoven in the nerve structure. Because of the risk of nerve damage during surgery, tumors that are not causing symptoms may be left alone.
Neurofibromas of the skull base can be approached directly by using the Endoscopic Endonasal Approach (EEA). This approach allows surgeons to see and access the tumor well without making incisions in the face or skull. EEA offers the benefits of no incisions to heal, no disfigurement to the patient, and a shorter recovery time.
At UPMC, we take a 360° Approach to treatment when evaluating each patient—looking at their conditions from every direction—to find the path that is least disruptive to the patient's brain, critical nerves, and ability to return to normal functioning. Our neurosurgical team may recommend a combination of surgical and non-surgical approaches to maximize the benefits of surgery while minimizing risks.
