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Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans
Daniel J Reilly1, Yew Loong Loo1, William M Alexander1
1From the Department of Plastic and Maxillofacial Surgery, Royal Children's Hospital.
Insights
Pediatric dermatofibrosarcoma protuberans (DFSP) is rare. Surgical excision achieved recurrence-free survival in all pediatric patients, highlighting the importance of complete tumor removal.
Area of Science:
- Oncology
- Pediatric Oncology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing fibrohistiocytic tumor originating in the dermis.
- Pediatric cases account for 6% of all DFSP diagnoses, with an incidence of 1 per million children.
Purpose of the Study:
- To review the management and outcomes of pediatric dermatofibrosarcoma protuberans (DFSP) cases.
- To evaluate the efficacy of surgical excision in achieving recurrence-free survival for pediatric DFSP.
Main Methods:
- Retrospective review of pediatric DFSP cases managed at a single institution over 23 years.
- Analysis of patient demographics, treatment modalities, and follow-up data.
- Surgical excision as the primary treatment approach for all patients.
Main Results:
- Seventeen pediatric patients (10 male, mean age 9.9 years) were included.
- Median follow-up was 29 months, with no observed recurrences.
- Complete surgical excision with clear margins (R0 resection) was achieved in all cases, with some requiring re-excision.
Conclusions:
- Pediatric DFSP can be effectively managed with surgical excision, leading to excellent outcomes.
- Multidisciplinary team management involving soft tissue tumor specialists, pathologists, and surgeons is crucial.
- Achieving R0 resection is key to ensuring recurrence-free survival in pediatric DFSP.
Background:
Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor of dermal origin. Six percent of all cases present in children, with a childhood incidence of 1 per million.
Methods:
This is a retrospective review of all cases of pediatric DFSP managed at a single institution over a 23-year period.
Results:
Seventeen patients (10 male; mean age, 9.9 years) were managed during the study period. The median follow-up was 29 months. All patients had surgical excision. Three patients required further excision to achieve uninvolved final margins. There were no recurrences observed.
Conclusions:
Pediatric DFSP should be managed by a soft tissue tumor multidisciplinary team, with experienced pathologists and reconstructive surgeons. Where R0 resections are obtained, patients can experience recurrence-free survival.
