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Additional Wide Resection of Infantile Dermatofibrosarcoma Protuberans after Unplanned Excision: A Case Report
Tomoharu Tanaka1, Robert Nakayama1, Tomoaki Mori1
1Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive intermediate soft tissue neoplasm that occurs in the dermis. DFSP generally occurs in young to middle-aged adults and rarely in infancy. Because of its extreme rarity, DFSP is difficult to diagnose and treat, especially when it occurs in infancy. In this paper, we reported a case of infantile DFSP in which we performed additional wide resection with a 3-cm horizontal margin for a mass that had previously undergone unplanned excision. No tumor recurrence has been seen for 3 years postoperatively. We suggest that the possibility of DFSP should always be considered when an enlarging superficial mass is identified on the trunk, even in an infant. Additionally, radical local treatment is as important for DFSP in infancy as it is for DFSP in adults, even after unplanned excision.
Insights
Dermatofibrosarcoma protuberans (DFSP) is rare in infants but requires prompt diagnosis. Radical surgical excision is crucial for successful treatment and preventing recurrence, even after initial unplanned removal.
Area of Science:
- Oncology
- Dermatology
- Pediatric Surgery
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue neoplasm originating in the dermis.
- While typically affecting adults, DFSP is exceptionally uncommon in infants, posing diagnostic and therapeutic challenges.
Observation:
- A case of infantile DFSP is presented, initially treated with unplanned excision.
- The infant presented with a superficial enlarging mass on the trunk.
Findings:
- Additional wide resection with a 3-cm margin was performed for the residual tumor.
- No recurrence of DFSP was observed for 3 years following the radical surgical intervention.
Implications:
- DFSP should be considered in the differential diagnosis of enlarging superficial masses in infants.
- Radical local treatment, including wide surgical margins, is essential for infantile DFSP, mirroring adult treatment protocols, even after prior incomplete excision.
