Dermatofibrosarcoma protuberans: Our experience of 59 cases

Area of Science:

• Oncology
• Dermatology
• Surgical Pathology

Background:

• Dermatofibrosarcoma protuberans (DFSP) is a rare, intermediate-grade soft tissue sarcoma originating in the skin.
• DFSP can infiltrate deep structures and has a high local recurrence rate after incomplete surgical removal.
• A specific chromosomal translocation, t(17;22)(q22;q13), is a key genetic driver in DFSP development.

Purpose of the Study:

• To evaluate the effectiveness of different surgical excision techniques for DFSP.
• To compare recurrence rates and the need for surgical revision between conventional and wide excision methods.
• To analyze the impact of surgical margins on DFSP treatment outcomes.

Main Methods:

• Retrospective analysis of 59 DFSP patient medical records from 1999 to 2011.
• Categorization of patients based on surgical treatment: conventional excision (n=13) and wide excision (n=46).
• Comparison of outcomes including tumor-free margins, surgical revisions, and local recurrence rates for each treatment group.

Main Results:

• Wide excision (78% of cases) resulted in 73% tumor-free margins and 0% recurrence.
• Conventional excision (22% of cases) yielded 5% tumor-free margins, with 14% requiring revision and 3% experiencing recurrence.
• Wide excision demonstrated a significantly lower rate of recurrence and revision compared to conventional excision.

Conclusions:

• Wide surgical excision is a more effective treatment for DFSP, achieving higher rates of tumor-free margins and eliminating local recurrence in this cohort.
• The findings highlight the importance of adequate surgical margins in DFSP management.
• Further research is needed to explore genotypic analysis of surgical margins to potentially identify microscopic disease in phenotypically healthy tissue.