Neurofibromatosis-1 Health Article

Treatment

There is no specific treatment for neurofibromatosis. Tumors that cause pain or loss of function may be removed. Tumors that have grown quickly should be removed promptly as they may become cancerous (malignant). Experimental treatments for severe tumors are under investigation.

Some children with learning disorders may need special schooling.

Support Groups

For more information and resources, contact the National Neurofibromatosis Foundation.

Expectations (prognosis)

If there are no complications, the life expectancy of people with neurofibromatosis is almost normal. With the right education and job expectations, people with neurofibromatosis can live a normal life.

Although mental impairment is generally mild, NF1 is a known cause of attention deficit hyperactivity disorder in a small number of patients. Learning disabilities are a common problem.

Some people are treated differently because they have hundreds of tumors on their skin.

Patients with neurofibromatosis have an increased chance of developing severe tumors. In rare cases, these can shorten a person's lifespan.

Complications

• Attention deficit hyperactivity disorder (ADHD)
• Blindness caused by a tumor in an optic nerve (optic glioma)
• Break in the leg bones that does not heal well
• Cancerous tumors
• Loss of function in nerves that a neurofibroma has put pressure on over the long term
• Pheochromocytoma, which causes very high blood pressure
• Regrowth of NF tumors
• Scoliosis, or curvature of the spine
• Tumors of the face, skin, and other exposed areas

Calling your health care provider

Call your health care provider if:

• You notice coffee-with-milk colored spots on your child's skin or any of the signs listed here.
• You have a family history of neurofibromatosis and are planning to have children, or would like to have your child examined.

Prevention

Genetic counseling is recommended for anyone with a family history of neurofibromatosis.

Annual eye exams are strongly recommended.

References

Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 2007;6:340-351.

Haslam RHA. Neurocutaneous Syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelston Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 596.