Charlotte B. van Lee, Wilner Christopher Kan, Sonia Gran, Antien Mooyaart, Marc A.M. Mureau, Hywel C. Williams, Rubeta Matin, Renate van den Bos, Loes M. Hollestein
DOI: 10.2340/00015555-3287

Dermatofibrosarcoma protuberans is a rare soft tissue tumour with a very low (< 0.5%) rate of metastasis. Rates of re-excision and recurrence were determined using data from the Netherlands Cancer Registry between 1989 and 2016. Of the 1,890 instances of dermatofibrosarcoma protuberans included, 87% were treated with excision, 4% with Mohs micrographic surgery, and 9% otherwise or unknown. Linked pathology data were retrieved for 1,677 patients. Half of all excisions (847/1,644) were incomplete and 29% (192/622) of all re-excisions were incomplete. The cumulative incidence of a recurrence was 7% (95% confidence interval (95% CI) 6–8) during a median follow-up of 11 years (interquartile range (IQR) 6–17). After Mohs micrographic surgery (n = 34), there were no recurrences during a median follow-up of 4 years (IQR 3–6). Due to the high rate of incomplete excisions and recurrences after excision, this study supports the European guideline, which recommends treating dermatofibrosarcoma protuberans with Mohs micrographic surgery in order to decrease the rate of recurrence.

Dermatofibrosarcoma protuberans is a rare soft tissue tumour, for which the quality of care is poorly studied. Rates of re-excision and recurrence were determined using data from the Netherlands Cancer Registry between 1989 and 2016. Of the 1,890 dermatofibrosarcoma protuberans included, 87% were treated with excision, 4% with Mohs micrographic surgery, and 9% otherwise or unknown. Half of all excisions (847/1,644) were incomplete and 29% (192/622) of all re-excisions were incomplete. Of the patients who received surgery, 7% needed multiple surgeries. Due to the high rate of incomplete excisions and recurrences after excision, this study supports the European guideline, which recommends treating dermatofibrosarcoma protuberans with Mohs micrographic surgery in order to increase the quality of care.