Epidemiology of Dermatofibrosarcoma Protuberans in Alberta, Canada, from 1988 to 2007

BACKGROUND: There have only been a few population-based, epidemiologic studies assessing dermatofibrosarcoma protuberans (DFSP). OBJECTIVE: To assess the epidemiology of DFSP in Alberta, Canada, over a 20-year period. METHODS: A population-based, retrospective analysis of all cases of DFSP in Alberta was conducted using data from the Alberta Cancer Registry. Sex-, age-, and anatomical location-specific incidence rates and trends were determined. RESULTS: The overall age-standardized incidence rate of DFSP remained stable at 0.93 per 100,000. DFSP prevalence was highest in individuals aged 20 to 39 (46.8%), followed by those aged 40 to 59 (34.0%), 60 and older (14.7%), and lastly younger than 20 (4.5%). The mean age at diagnosis was 41.1 (women) and 43.1 (men). The incidence of DFSP in men and women has shown a dramatic shift such that incidence in women has increased 3.2% per year, whereas in men it has decreased 2.7% per year. In women, DFSP incidence increased on the trunk and decreased on the upper extremities. CONCLUSION: The age-standardized incidence of DFSP observed is nearly twice as high as previously reported and has remained stable. The incidence is increasing in women and decreasing in men. DFSP primarily affects young to middle-aged adults and most commonly presents on the trunk.