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Neurofibromatosis.

Authors
Type
Published Article
Journal
Clinics in plastic surgery
Publication Date
Volume
14
Issue
2
Pages
315–325
Identifiers
PMID: 3107867
Source
Medline

Abstract

Neurofibromatosis (NF) is an inherited disorder characterized by the development of a wide variety of clinical manifestations, including characteristic "freckle-like" pigmentations (cafe au lait spots) that develop in infancy, followed by skin tumors that may vary widely in size, number, and distribution. In addition to skin tumors, bone, neurologic, and endocrine abnormalities are common. NF is recognized in eight different types, with clinical heterogeneity being the hallmark of this disease. Malignant degeneration of the tumor to neurofibrosarcoma is rare. Early diagnosis, genetic counseling, treatment of symptoms, and appropriate surgery are the tools available for management of NF patients. Surgical procedures generally involve judicious subtotal resection, combined with reconstructive efforts aimed at functional improvement and cosmesis.

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