Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years

Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and All...

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Autores principales: Wollina, Uwe, Langner, Dana, Schönlebe, Jacqueline, França, Katlein, Lotti, Torello, Tchernev, Georgi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Republic of Macedonia 2018
Materias:
Clinical Science
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816309/
https://www.ncbi.nlm.nih.gov/pubmed/29483976
http://dx.doi.org/10.3889/oamjms.2018.030
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author Wollina, Uwe
Langner, Dana
Schönlebe, Jacqueline
França, Katlein
Lotti, Torello
Tchernev, Georgi
author_facet Wollina, Uwe
Langner, Dana
Schönlebe, Jacqueline
França, Katlein
Lotti, Torello
Tchernev, Georgi
author_sort Wollina, Uwe
collection PubMed
description Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments.
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spelling pubmed-58163092018-02-26 Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years Wollina, Uwe Langner, Dana Schönlebe, Jacqueline França, Katlein Lotti, Torello Tchernev, Georgi Open Access Maced J Med Sci Clinical Science Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments. Republic of Macedonia 2018-01-10 /pmc/articles/PMC5816309/ /pubmed/29483976 http://dx.doi.org/10.3889/oamjms.2018.030 Text en Copyright: © 2018 Uwe Wollina, Dana Langner, Jacqueline Schönlebe, Katlein França, Torello Lotti, Georgi Tchernev. http://creativecommons.org/licenses/CC BY-NC/4.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).
spellingShingle Clinical Science
Wollina, Uwe
Langner, Dana
Schönlebe, Jacqueline
França, Katlein
Lotti, Torello
Tchernev, Georgi
Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title_full Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title_fullStr Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title_full_unstemmed Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title_short Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years
title_sort dermatofibrosarcoma protuberans: retrospective single center analysis over 16 years
topic Clinical Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816309/
https://www.ncbi.nlm.nih.gov/pubmed/29483976
http://dx.doi.org/10.3889/oamjms.2018.030
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