title: Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: *DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, FL †New York Medical College, New York, NY ‡Division of Plastic, Aesthetic, and Reconstructive Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, FL.
authors.forename: Gustavo A
authors.initials: GA
authors.lastname: Rubio
authors.forename: Andrea
authors.initials: A
authors.lastname: Alvarado
authors.forename: David J
authors.initials: DJ
authors.lastname: Gerth
authors.forename: Jun
authors.initials: J
authors.lastname: Tashiro
authors.forename: Seth R
authors.initials: SR
authors.lastname: Thaller
mesh_terms: D000293:Adolescent; D003131:Combined Modality Therapy; D018223:Dermatofibrosarcoma / Q000453:epidemiology* / Q000628:therapy; D005121:Extremities; D005260:Female; D006257:Head; D006801:Humans; D015994:Incidence; D008297:Male; D009333:Neck; D011379:Prognosis; D012878:Skin Neoplasms / Q000453:epidemiology* / Q000628:therapy; D060726:Torso; D014481:United States / Q000453:epidemiology; D055815:Young Adult