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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Detalles Bibliográficos
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
Descripción
Sumario: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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