Dermatofibrosarcoma Protuberans Re-excision and Recurrence Rates in the Netherlands Between 1989 and 2016

Abstract

SIGNIFICANCEDermatofibrosarcoma 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.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 (CI) 6-8) during a median followup 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.

Mohs micrographic surgery in dermatofibrosarcoma protuberans: Rate and risk factors for recurrence in a prospective cohort study from the Spanish Registry of Mohs Surgery (REGESMOHS) and review of the literature

“…In agreement with other studies, the majority of recurrent DFSP occurred among young people (mean age of 46.6) and the most common locations were trunk and extremities 1,2,6,11 . A slight female predominance for recurrence has been constantly reported, 3,6,8,11 similar to what we found. In our study, recurrent tumors were characterized by larger diameter (>10 mm) and deeper invasion.…”

“…Data from the Netherlands Cancer Registry were published by Van Lee et al 11 ; they analysed DFSP re‐excisions and recurrences from 1890 cases included in the registry from 1989 to 2016. Despite being a representative cohort, only 4% were treated with MMS.…”

Dermatofibrosarcoma protuberans: slowly gathering evidence for optimal treatment of a slow‐growing tumour

“…Almost 20% of standard excisions were incomplete at the first attempt. Recurrence rates in the current study were lower than previously reported (1.2% vs. 7%) 1,4 . However, this is probably an underestimation as the median follow‐up time of treated DFSPs in this study was very short (2.2 years).…”

A 10‐year review of surgical management of dermatofibrosarcoma protuberans*

“…Two large population‐based studies for DFSP from the USA 1,3 have used cancer registries providing large datasets, but individual case‐specific data are lacking. A more recent retrospective review using data from the Netherlands Cancer Registry with linked pathology demonstrated high rates of incomplete surgical excisions 31 . Variations in healthcare systems, costs and accessibility to treatment in different countries may affect the external validity of these databases.…”