Diffuse plexiform neurofibroma of the back: report of a case.

Abstract

Neurofibromatosis type 1 is an autosomal dominant disorder affecting the ras proto-oncogene. It is characterized by the overgrowth of nervous tissue and skin discoloration. While it is associated with a variety of phenotypic presentations, it is the plexiform variant that is particular concerning, as it can become extremely disfiguring and has a propensity for malignant degeneration. A case of a Pacific Islander with a large plexiform type 1 neurofibroma is presented. The patient was ultimately treated with surgical resection, negative pressure wound therapy, and split-thickness skin grafting with good results. A review of the literature concerning the diagnosis and treatment of neurofibromatosis is included.