title: Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes.
abstract: Little is known about the incidence and management of dermatofibrosarcoma protuberans (DFSP) in children. We conducted a retrospective review of all patients younger than 18 years of age treated for DFSP over a period of 11 years (2000-2011) in Madrid, Spain. The sample consisted of 13 children. The average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases per million person-years (95% confidence interval 0.55, 1.73). Sites of involvement were diverse, with 15.3% of tumors found in acral locations. The median tumor size was 3.5 cm × 3 cm and the median time from apparent onset to diagnosis was 36 months. Histopathologic examination revealed conventional (77.0%), pigmented (15.4%), and myxoid (7.6%) variants. The mitotic index was consistently <5 per 10 high-power fields. All lesions were removed using surgical excision. One patient developed a local recurrence because of initial affected margins; none developed metastases. The median duration of clinical follow-up was 70.5 months. This study estimated the average annual incidence rate of DFSP in a population of patients younger than 18 years and reviewed the experience of several hospitals in the management of this tumor.
authors.affiliation: Department of Dermatology, Hospital Infanta Leonor, Madrid, Spain.
authors.forename: Marta
authors.initials: M
authors.lastname: Valdivielso-Ramos
authors.forename: Antonio
authors.initials: A
authors.lastname: Torrelo
authors.forename: Minia
authors.initials: M
authors.lastname: Campos
authors.forename: Marta
authors.initials: M
authors.lastname: Feito
authors.forename: Reyes
authors.initials: R
authors.lastname: Gamo
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Rodriguez-Peralto
mesh_terms: D000293:Adolescent; D002648:Child; D018223:Dermatofibrosarcoma / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D013030:Spain / Q000453:epidemiology