Neurofibromas consist of a well circumscribed proliferation of schwann cells. The presence of cafe au lait spots on the skin are characteristic of neurofibromatosis, type1. NF-1 is an autosomal dominant inherited disease attributed to a genetic mutation on the long arm of chromosome 17. It has also been reported frequently in affected young, school-aged children. The second most common characteristic of NF-1 is multiple neurofibromas.
Dermal neurofibromas can be solitary or multifocal and occur anywhere on the body.
Subcutaneous neurofibromas are palpable masses that occur along the peripheral nerves beneath the skin and in the oral cavity. The intraoral neurofibromas are most commonly found on the tongue, buccal mucosa, edentulous alveolar ridges, palate, and floor of mouth. Impaction of permanent teeth has also been associated with some intraoral neurofibromas. Plexiform neurofibromas are large, disfiguring, swellings in the face, neck, and extremities that can result in complications if left untreated. Lisch nodules, are well-defined, pigmented nodules on the iris.