How is neurofibromatosis type 1 (NF1) managed?

At this time, NF1 can’t be cured. However, symptoms can be followed and managed. Because the symptoms differ between people, the management and treatments also may differ.

  • If you have NF1, you should have at least one regular medical follow-up examination every year, along with an ophthalmologic (eye) examination every year. In addition, you should get your blood pressure taken at least every year to see if the renal (kidney) arteries are narrowing.
  • Surgery can remove disfigurements caused by neurofibromas.
  • The cholesterol drug lovastatin is being used to treat learning disabilities and ADHD.
  • If you have NF1, you might find it useful to get psychological and social support, and possibly genetic counseling.

How are neurofibromatosis type 1 (NF1) tumors treated?

Neurofibromatosis type 1 produces tumors of the skin, internal organs, and nerves because normal genes that control cell growth are affected. These tumors are treated as follows:

  • Dermal neurofibromas: Surgery is usually recommended if these skin tumors are painful or irritating.
  • Plexiform neurofibromas: These tumors are deeper than dermal neurofibromas and affect nerves. Your healthcare provider may choose to monitor these tumors are and not treated them, but chemotherapy and surgery are options.
  • Malignant peripheral nerve sheath tumors (MPNST): These tumors develop from plexiform lesions. The diagnosis is made by surgery. Treatment involves surgery, radiation therapy and chemotherapy.
  • Gastrointestinal stromal tumors (GIST): These tumors develop in the small intestine. They are treated with surgery. Several chemotherapy trials are under way.
  • Optic nerve glioma and astrocytoma: These tumors develop in the eye (optic nerve glioma) and brain (astrocytoma). The grade of the tumor usually defines how fast it grows and how difficult it is to treat. Grade 4 astrocytomas are the most aggressive brain tumors and the most difficult to treat. If you’re diagnosed with a brain tumor, you should seek a specialist in neuro-oncology.

Last reviewed by a Cleveland Clinic medical professional on 12/15/2020.

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