Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years

Authors

  • Uwe Wollina Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen
  • Dana Langner Städtisches Klinikum Dresden - Department Dermatology and Allergology, Dresden
  • Jacqueline Schönlebe Städtisches Klinikum Dresden - Institute of Pathology "Georg Schmorl", Dresden
  • Katlein França 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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Published

2018-01-10

How to Cite

1.
Wollina U, Langner D, Schönlebe J, França K, Lotti T, Tchernev G. Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years. Open Access Maced J Med Sci [Internet]. 2018 Jan. 10 [cited 2021 Apr. 11];6(1):35-7. Available from: https://oamjms.eu/index.php/mjms/article/view/oamjms.2018.030

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