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Informative Article | Orthopaedic Surgery | Malaysia | Volume 12 Issue 12, December 2023

Dermatofibrosarcoma Protuberans Masquerading as Vascular Anomaly: A Diagnostic Challenge in Pediatric Patients

T. L. Tie | D. Suryasmi

Abstract: Soft tissue sarcoma in pediatric patients, particularly dermatofibrosarcoma protuberans (DFSP), poses a diagnostic challenge due to their clinical resemblance to other vascular lesions. This retrospective study analyses pediatric sarcoma cases, including 4 misdiagnosed DFSP cases initially treated as hemangiomas. Patient 1, a 10-year-old boy, exhibited aggressive DFSP progression despite surgical intervention, leading to a fatal outcome. Patient 2, a 10-year-old girl, experienced DFSP recurrence after initial surgery, declining further intervention and remaining alive with the disease. Patient 3, a 5-month-old boy underwent multiple surgeries due to recurrent DFSP, ultimately leading to a modified Chopart amputation. These cases highlight the critical importance of accurate diagnosis through biopsy before surgical intervention in pediatric vascular lesions. Enhanced clinical awareness and vigilance are imperative to differentiate DFSP from other conditions, ensuring appropriate treatment strategies and improved patient outcomes.

Keywords: Soft tissue sarcoma, Dermatofibrosarcoma protuberans, Vascular anomaly

Edition: Volume 12 Issue 12, December 2023,

Pages: 238 - 242