Dermatofibrosarcoma Protuberans in Pediatric Patients

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor. In children, there are acquired and congenital presentations. Due to clinical similarities with other conditions, diagnosis may be delayed. Objective: To review the clinical characteristics and treatment of DFSP in pediatric patients. Methods: A retrospective chart review was performed from January 2002 to September 2012. Inclusion criteria were patients under 18 years of age with a histopathologic diagnosis of DFSP. Data on demographics, clinical characteristics, treatments, and outcomes were collected. Results: Information was gathered from 17 patients; 9 (53%) were female. Congenital lesions were reported in 7 patients. The mean delay of diagnosis was 5.7 years; the most common anatomic location was the trunk in 8 of 17 (47%) cases. Treatment options included wide local surgery, Mohs surgery, and imatinib mesylate. Conclusion: A detailed medical history and identification of the natural course of common conditions seen in pediatric patients are important to identify less common lesions and to suspect DFSP.