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Pediatric Dermatofibrosarcoma Protuberans in Madrid, Spain: Multi‐institutional Outcomes
Author(s) -
ValdivielsoRamos Marta,
Torrelo Antonio,
Campos Minia,
Feito Marta,
Gamo Reyes,
RodriguezPeralto Jose Luis
Publication year - 2014
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12371
Subject(s) - dermatofibrosarcoma protuberans , medicine , incidence (geometry) , confidence interval , sarcoma , retrospective cohort study , population , surgery , pediatrics , dermatology , pathology , physics , environmental health , optics
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.