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[Rare form of a segmental neurofibromatosis with giant plexiform neurofibroma].
Hautarzt. 2019 Aug; 70(8):638-640.H

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.

Authors+Show Affiliations

Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland. robin.reschke@medizin.uni-leipzig.de.Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.

Pub Type(s)

Case Reports
Journal Article

Language

ger

PubMed ID

31076816

Citation

Reschke, Robin, et al. "[Rare Form of a Segmental Neurofibromatosis With Giant Plexiform Neurofibroma]." Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, vol. 70, no. 8, 2019, pp. 638-640.
Reschke R, Kadlecova M, Grunewald S, et al. [Rare form of a segmental neurofibromatosis with giant plexiform neurofibroma]. Hautarzt. 2019;70(8):638-640.
Reschke, R., Kadlecova, M., Grunewald, S., & Ziemer, M. (2019). [Rare form of a segmental neurofibromatosis with giant plexiform neurofibroma]. Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, 70(8), 638-640. https://doi.org/10.1007/s00105-019-4419-3
Reschke R, et al. [Rare Form of a Segmental Neurofibromatosis With Giant Plexiform Neurofibroma]. Hautarzt. 2019;70(8):638-640. PubMed PMID: 31076816.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Rare form of a segmental neurofibromatosis with giant plexiform neurofibroma]. AU - Reschke,Robin, AU - Kadlecova,Marketa, AU - Grunewald,Sonja, AU - Ziemer,Mirjana, PY - 2019/5/12/pubmed PY - 2019/9/26/medline PY - 2019/5/12/entrez KW - Excision KW - Mosaicism KW - Mutation KW - Neurodegenerative diseases KW - Peripheral nervous system neoplasms SP - 638 EP - 640 JF - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JO - Hautarzt VL - 70 IS - 8 N2 - 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. SN - 1432-1173 UR - https://www.unboundmedicine.com/medline/citation/31076816/[Rare_form_of_a_segmental_neurofibromatosis_with_giant_plexiform_neurofibroma]_ L2 - https://dx.doi.org/10.1007/s00105-019-4419-3 DB - PRIME DP - Unbound Medicine ER -