Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years

Uwe Wollina; Dana Langner; Jacqueline SchÃ¶nlebe; Katlein FranÃ§a; Torello Lotti; Georgi Tchernev

doi:10.3889/oamjms.2018.030

Authors

Uwe Wollina StÃ¤dtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen
Dana Langner StÃ¤dtisches Klinikum Dresden - Department Dermatology and Allergology, Dresden
Jacqueline SchÃ¶nlebe StÃ¤dtisches Klinikum Dresden - Institute of Pathology "Georg Schmorl", Dresden
Katlein FranÃ§a Department of Dermatology and Cutaneous Surgery, Department of Psychiatry & Behavioral Sciences; Institute for Bioethics and Health Policy, University of Miami Miller School of Medicine, Miami, FL
Torello Lotti University of Rome, Institute of Deramtology, Rome
Georgi Tchernev Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, Sofia; Onkoderma Policlinic for Dermatology and Dermatologic Surgery, Sofia

DOI:

https://doi.org/10.3889/oamjms.2018.030

Keywords:

Sarcoma, dermatofibrosarcoma protuberans, CD34, surgery, outcome, targeted therapy

Abstract

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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