Neurofibroma Neurofibromatosis Wheeless

Description: Discussion: - neurofibroma is a benign tumor of neural origin; - has an autosomal dominant trait w/ variable penetrance & high rate of spontaneous mutation; - it is the most common single gene disorder (1 in 3000); - presentation: - neurofibromas are most commonly found in skin and subQ tissue or associated w/ nerve fibers; - tumors may be solitary or multiple (multiple neurofibromas are most common); - may affect patients of any age; - most neurofibromas present as an active stage 2 tumors, but aggresive stage 3 infiltratrive forms may be seen occasionally; - long standing neurofibromatosis may undergo malignant transformation to neurosarcoma, which is often heralded by a new onset neurologic deficit;