Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population

Background: Dermatofibrosarcoma protuberans (DFSP) is a low-grade soft tissue sarcoma. In the pediatric population, DFSP is exceedingly rare. Aim of this study was to describe the epidemiology and clinical outcomes in a large pediatric cohort.

Methods: Surveillance, Epidemiology, and End Results (SEER) database (1973-2010) was analyzed for all patients with dermatofibrosarcoma occurring in patients <20 years of age. Data were extracted based on age, gender, race, anatomic site, histology, stage, treatment modalities, and survival. Incidence rates were standardized to the 2000 US population.

Results: A total of 451 patients were identified. Overall annual incidence was 0.10 per 100,000. Incidence was highest among black children and adolescents (ages 15 to 19 years). Trunk was most common site, followed by extremities. Head and neck region was least common site (P < 0.05). Majority (54%) of patients presented with localized disease. Overall, 95% underwent surgery. Only 2.2% were treated with perioperative radiation therapy. Overall prognosis was favorable with 5-year overall survival (OS) of 100%, 15-year OS of 98%, and 30-year OS of 97%. Median survival was 117 months. Male patients had lower 15- and 30-year OS compared with females (P < 0.05).

Conclusion: Pediatric DFSP has lower incidence but similar clinical characteristics to adults. Incidence is higher in black children and in the trunk region. While prognosis is favorable, male sex is associated with decreased OS.