Dermatofibrosarcoma Protuberans in Pediatric Patients: A Report of 17 Cases. Issue 3 (May 2014)

• Record Type: Journal Article
• Authors: Posso-De Los Rios, Claudia J.; Lara-Corrales, Irene; Ho, Nhung
• Is Part Of: Journal of cutaneous medicine and surgery. Volume 18:Issue 3(2014)
• Page Start: 180
• Page End: 185
• Publication Date: 2014-05
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Treatment -- Periodicals; Skin -- Surgery -- Periodicals; Dermatology -- Canada -- Periodicals; Skin -- Diseases -- Treatment -- Canada -- Periodicals; Skin -- Surgery -- Canada -- Periodicals; 616.5005
• DOI: 10.2310/7750.2013.13099
• Languages: English
• ISSNs: 1203-4754

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.