Dermatofibrosarcoma Protuberans

2017 ◽  
Author(s):  
Benjamin Deschner ◽  
Jeffrey Wayne
Keyword(s):  
Skin Cancer ◽  
Local Recurrence ◽  
Distant Metastasis ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Micrographic Surgery ◽  
Complete Surgical Resection ◽  
Cutaneous Tumor ◽  
Almost All ◽  
Aggressive Variant

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.   This review contains 5 figures, 4 tables and 28 references Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision

Dermatofibrosarcoma Protuberans

2017 ◽  
Author(s):  
Benjamin Deschner ◽  
Jeffrey Wayne
Keyword(s):  
Skin Cancer ◽  
Local Recurrence ◽  
Distant Metastasis ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Micrographic Surgery ◽  
Complete Surgical Resection ◽  
Cutaneous Tumor ◽  
Almost All ◽  
Aggressive Variant

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.   This review contains 5 figures, 4 tables and 28 references Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision

scholarly journals Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years

2018 ◽  
Vol 6 (1) ◽  
pp. 35-37
Author(s):  
Uwe Wollina ◽  
Dana Langner ◽  
Jacqueline Schönlebe ◽  
Katlein França ◽  
Torello Lotti ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
High Risk ◽  
Local Recurrence ◽  
Three Dimensional ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Metastatic Spread ◽  
Single Center ◽  
Emerging Treatments ◽  
Margin Control

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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