Plexiform neurofibroma

Plexiform neurofibroma is a type of tumor that is associated with Neurofibromatosis type I (NF1), a genetic disorder. These tumors are benign, but they can cause significant complications due to their size and location.

Overview

Plexiform neurofibromas are a hallmark of NF1. They are present in approximately 30% of individuals with this disorder. These tumors can occur anywhere in the body, but they are most commonly found in the skin, subcutaneous tissues, and peripheral nerves. They can also occur in the brain and spinal cord.

Symptoms

The symptoms of plexiform neurofibroma can vary widely, depending on the size and location of the tumor. Common symptoms include:

• Pain
• Numbness or tingling
• Weakness
• Skin discoloration
• Deformity or disfigurement

Diagnosis

The diagnosis of plexiform neurofibroma is typically made based on clinical findings and imaging studies. Magnetic resonance imaging (MRI) is the most commonly used imaging modality for this purpose. Genetic testing can also be used to confirm the diagnosis of NF1.

Treatment

The treatment of plexiform neurofibroma is primarily surgical. However, surgery is often challenging due to the diffuse nature of these tumors. Other treatment options include chemotherapy and radiation therapy. In some cases, targeted therapy with drugs that inhibit the growth of tumor cells may be used.

Prognosis

The prognosis for individuals with plexiform neurofibroma is variable. Some individuals may have a relatively normal lifespan, while others may experience significant morbidity and mortality due to complications of the tumor.

See also

• Neurofibromatosis
• Benign tumor
• Genetic disorder

Plexiform neurofibroma Resources
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