Surgical Management of Pediatric Dermatofibrosarcoma Protuberans: About Two Challenging Case Reports

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare cutaneous tumor in children, marked by local aggressiveness, slow growth, high recurrence rate, and low metastatic potential. Its prevalence is often underestimated in children due to its slow growth and frequent misdiagnosis. Diagnosing DFSP can be challenging due to nonspecific symptoms. While most cases present as nodular lesions on the trunk or proximal extremities, some lesions, such as atrophic plaques or sclerotic nodular plaques, can mimic vascular malformations and confuse clinicians. Histologic and immunohistochemical studies are essential for definitive diagnosis. The treatment of choice is complete surgical resection with wide margins to reduce the risk of recurrence. We report two pediatric DFSP cases on the trunk, including one mimicking vascular malformations. Both cases had successful 4 cm margin resections, with no recurrences observed after 6 months and 2 years of follow-up, respectively. Continuous surveillance will be maintained for at least 5 years.