BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with an infiltrative growth pattern that makes it challenging to clear margins. High quality data regarding DFSP natural history, management, and outcomes are limited. METHODS: Data were retrospectively collected for adult DFSP patients who underwent resection at 10 institutions in eight countries. Demographics, tumor characteristics, treatment strategies, and outcomes were analyzed. RESULTS: Analysis included 347 patients consisting of young (median, 42 years), White (76.2%), males (54.2%) with truncal lesions (57.3%). The majority (76.8%) were symptomatic at presentation. Preoperative imaging was used in 55.9% of cases. Diagnosis was established with excisional biopsy in 50.9% versus incisional biopsy in 25.0% of cases. Despite planned margins of >1.0 cm in 67.4% of cases, only 69.0% of patients achieved R0 resection. Twenty-two percent of patients underwent at least one re-excision. R0 resection was achieved at a second procedure in 80.2% and a third procedure in 86.2%. Ultimately, R0 resection was feasible in 89.5% of all patients. Fibrosarcomatous transformation (FST) was observed in 12.6%. In total, 6.6% (N = 23) recurred (17 local, six distant). Of the six distant recurrences, 50.0% had FST. With a median follow-up of 47.0 months, disease-specific survival rate was 98.8%. In multivariable analysis, R0 margins at index resection were associated with wider circumferential margins and non-FST histology. CONCLUSIONS: In this international, multicenter collaborative, DFSP practice patterns were heterogeneous but achieved favorable recurrence rates and survival. Multiple excisions to clear margins remain commonplace and can inform future efforts to optimize margin selection.

Department of General Surgery Temple University Hospital Philadelphia Pennsylvania USA

Department of Medical Oncology Inselspital Bern University Hospital Univesity of Bern Bern Switzerland

Department of Musculoskeletal Oncology National Cancer Center Hospital Tokyo Japan

Department of Plastic and Hand Surgery Inselspital Bern University Hospital Freiburgstrasse Switzerland

Department of Surgery Duke University Hospital Durham North Carolina USA

Department of Surgery Masaryk Memorial Cancer Institute Brno Zluty kopec Czech Republic

Department of Surgical Oncology University Hospitals Leuven Leuven Belgium

Division of Biostatistics and Bioinformatics Fox Chase Cancer Center Philadelphia Pennsylvania USA

Division of Medical Oncology Fox Chase Cancer Center Philadelphia Pennsylvania USA

Division of Surgery Institute of Oncology Ljubljana Ljubljana Slovenia

Division of Surgical Oncology Emory University Hospital Atlanta Georgia USA

Division of Surgical Oncology Fox Chase Cancer Center Philadelphia Pennsylvania USA

Division of Surgical Oncology Ottawa Hospital Ottawa Ontario Canada

Sarcoma Unit Queen Elizabeth Hospital Birmingham United Kingdom

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