[Rare form of a  segmental neurofibromatosis with giant plexiform neurofibroma].

[Rare form of a segmental neurofibromatosis with giant plexiform neurofibroma]. Hautarzt. 2019 May 10;: Authors: Reschke R, Kadlecova M, Grunewald S, Ziemer M Abstract The case of segmental neurofibromatosis (NF) with a monstrous plexiform neurofibroma in a 53-year-old female patient is described. Segmental NF is a rare form of NF, which is caused by a postzygotic mutation in the NF1 gene. In this mosaic form the typical cutaneous symptoms of NF are limited to certain unilateral dermatomes. Plexiform neurofibromas are clinically and histologically in contrast to locally delimited neurofibromas. They involve the catchment area of a peripheral nerve, affect many fascicles and nerve branches, do not respect growth limits and spread in a reticulated fashion. Plexiform neurofibromas can become malignant. In the presented case large parts of the monstrous plexiform cutaneous neurofibroma were excised and the patient did not wish any further measures to be carried out for the time being. PMID: 31076816 [PubMed - as supplied by publisher]
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - Category: Dermatology Tags: Hautarzt Source Type: research