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CHAPTER 22
Neurofibromatosis
CHAPTER OBJECTIVES
- Describe the etiology and various forms of neurofibromatosis.
- Review the genetics associated with neurofibromatosis types 1 and 2.
- Provide diagnostic criteria for neurofibromatosis to assist the primary care provider.
- Detail current medical management options and recommendations for neurofibromatosis.
KEY TERMS
Tumor suppressor gene
A neurofibroma is defined as a benign, encapsulated tumor resulting from proliferation of Schwann cells that are of ectodermal (neural crest) origin and that form a continuous envelope around each nerve fiber of peripheral nerves. The autosomal dominant genetic disorder known as neurofibromatosis (NF) causes such tumors to grow on the sheaths of the nerves anywhere in the body at any time. This disorder affects 1 in 3,000–4,000 males and females of all races and ethnic groups worldwide and is one of the most common genetic disorders in the United States.
KEY TERM
Neurofibroma: a benign, encapsulated tumor resulting from proliferation of Schwann cells that are of ectodermal (neural crest) origin and that form a continuous envelope around each nerve fiber of peripheral nerves.
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