Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. OBJECTIVE The authors sought to establish a comprehensive report on the incidence of and survival from primary DFSP. METHODS The authors used data from the 18 registries of the Surveillance, Epidemiology, and End Results Program from 2000 to 2010. RESULTS Overall incidence was 4.1 per million person-years and steady over the decade. Trunk was the most common anatomic site except in older men. Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. CONCLUSION This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. The epidemiologic profile of DFSP differs from most skin cancers. Incidence is stable and highest among women and blacks. Worse survival is associated with increased age, male sex, black race, and anatomic location of the limbs and head.