Ipsilateral Sphenoid Wing Dysplasia, Orbital Plexiform Neurofibroma and Fronto-Parietal Dermal Cylindroma in a Patient with Segmental Neurofibromatosis

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal-dominant inherited disease affecting various parts of the body. Penetrance is almost complete but the phenotype varies considerably. Recently, mosaicism in NF1 has come into the focus of scientific interest. This report refers to a patient with orbitotemporal-confined neurofibromatosis who developed a rare skin tumor in the region.

Bibliographical data

Original languageEnglish
ISSN0250-7005
Publication statusPublished - 12.2015
PubMed 26637901