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What Is a Neurofibroma?
A neurofibroma is a benign tumor that develops from the Schwann cells surrounding nerve fibers. These tumors can infiltrate the nerve, disrupting the protective sheaths of individual fibers.
Neurofibromas may appear as a single tumor or in clusters, with multiple neurofibromas often being associated with neurofibromatosis (NF), a genetic disorder also known as von Recklinghausen disease.
Spinal neurofibromas are rare, comprising approximately 3 percent of all spinal tumors. When located in the spine, these tumors can put pressure on the spinal cord or nerve roots, leading to neurological symptoms.
What are the types of neurofibroma?
According to the National Institute of Neurological Disorders and Stroke, neurofibromas are categorized into three main types:
- Cutaneous neurofibromas — Small, benign tumors that form on or just under the skin. They are usually harmless and may appear anywhere on the body.
- Subcutaneous neurofibromas — Deeper tumors located beneath the skin, which may cause discomfort or pain if they press on nearby nerves.
- Plexiform neurofibromas — More complex tumors that grow along the length of nerves, often involving multiple nerve bundles. They are typically associated with neurofibromatosis type 1 (NF1) and, in about 10 percent of cases, may become malignant (cancerous).
Can neurofibromas become malignant?
Although most neurofibromas remain benign, certain types, particularly plexiform neurofibromas, have the potential to become malignant.
Malignant peripheral nerve sheath tumors (MPNSTs), which arise from neurofibromas, require aggressive treatment. Rarely, malignant gliomas (a type of brain cancer) can develop in adults with neurofibromatosis.
What causes neurofibroma?
The exact cause of neurofibromas is unknown, but research suggests that genetic mutations affecting cell growth in the nervous system play a key role.
What are neurofibroma risk factors?
The main primary risk factor for neurofibromas is neurofibromatosis (NF), a progressive, hereditary disorder that leads to tumor formation on nerves throughout the body, including the spine.
How common are neurofibromas?
Neurofibroma affects people of all genders and ethnic backgrounds.
Neurofibromatosis, the genetic condition responsible for multiple neurofibromas, is one of the most common genetic disorders in the U.S, occurring in one of every 2,500 to 3,000 births and affecting more than 120,000 Americans.
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What Are the Signs and Symptoms of a Neurofibroma?
Symptoms of neurofibromas depend on the location, size, and growth rate of the tumor. Although many neurofibromas cause no symptoms, spinal neurofibromas can lead to:
- Loss of bladder or bowel control (if the tumor compresses your spinal cord).
- Numbness or decreased sensation in affected areas.
- Persistent back pain that worsens over time.
- Weakness or paralysis in areas controlled by affected nerves.
When should I see a doctor about my neurofibroma symptoms?
If you experience neurofibroma symptoms — especially progressive weakness, numbness, or loss of bladder/bowel control — seek medical attention right away. Early diagnosis can help prevent complications and improve treatment outcomes.
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How Do You Diagnose a Neurofibroma?
Your doctor will perform a physical exam and ask about your symptoms.
During the exam, your doctor may apply light pressure to your tumor, which may produce an electric shock-like sensation. If your tumor affects motor or sensory nerves, corresponding functions may be impaired.
Diagnostic tests for neurofibroma include:
- Biopsy — A sample of the tumor taken during surgery to confirm the diagnosis and rule out malignancy.
- Genetic testing — In cases where neurofibromatosis is suspected, genetic testing may be recommended.
- MRI — Provides detailed images of your spine to detect neurofibromas.
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How Do You Treat a Neurofibroma?
Not all neurofibromas require treatment. If your tumor is small, slow-growing, and asymptomatic, your doctor may recommend a watch-and-wait approach with regular monitoring.
Medicine to treat neurofibromas
If the tumor compresses your spinal cord, corticosteroids may be administered to reduce swelling and inflammation.
Surgery for neurofibromas
Surgical removal is an option for neurofibromas that are causing pain, neurological symptoms, or other complications. Traditional open surgery may be required for deeply embedded tumors. However, because surgery carries a risk of nerve damage, treatment is typically reserved for neurofibromas that are causing symptoms.
Stereotactic radiosurgery (SRS)
Some spinal neurofibromas may be treated with SRS, a noninvasive procedure using highly focused radiation beams to target and destroy tumors. SRS minimizes damage to surrounding healthy tissue and has fewer side effects when compared to traditional surgery.
Radiation therapy for neurofibromas
If tumors cannot be completely removed during surgery, radiation therapy may help shrink them and relieve spinal cord compression.
What is the prognosis after neurofibroma treatment?
The prognosis for neurofibromas depends on their location, size, and growth behavior. Most people with benign neurofibromas experience good long-term outcomes, particularly if the tumors do not become malignant. Regular follow-up care with imaging and neurological evaluations can help monitor for any changes or complications.
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Why Choose UPMC for Neurofibroma Care?
UPMC is nationally recognized for excellence in spine care. We offer comprehensive care from diagnosis through treatment and beyond. Our team offers the most advanced nonsurgical and surgical care for a full range of spinal disorders and is engaged in pioneering research to develop novel treatment options.
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