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Neurofibroma and Neurofibromatosis

What is Neurofibroma?

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. Neurofibromas typically are painless, slow-growing masses, and may cause no symptoms.

At UPMC, the preferred surgical treatment for neurofibroma of the skull base is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. Benefits of EEA treatment include:

  • No incisions to heal
  • No disfigurement
  • Faster recovery time

Diagnosing Neurofibroma

To diagnose neurofibroma, your doctor will:

  • Perform a physical exam
  • Ask about any symptoms you are experiencing

Symptoms of neurofibroma and neurofibromatosis

Symptoms depend on the location and size of the tumor. Neurofibromas typically are painless and slow-growing.

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.

Imaging and radiology tests to diagnose neurofibroma and neurofibromatosis

Your doctor may order an MRI to diagnose neurofibroma because it has a characteristic appearance on the scan.

A biopsy of the tumor taken during surgery enables positive diagnosis.

Neurofibroma Treatment

Surgery is the treatment of choice for neurofibromas; however, surgery 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.

Surgery: Endoscopic Endonasal Approach (EEA)

Neurofibromas of the skull base can be approached directly by using the Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the neurofibroma through the nose and nasal cavities.

EEA treatment offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.

UPMC's neurosurgical team comprehensively evaluates your condition, looking at your tumor from every direction. We will use the treatment path that is least disruptive to your brain, critical nerves, and ability to return to normal functioning.

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