Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes
Marta Valdivielso-Ramos1, Antonio Torrelo, Minia Campos
1Department of Dermatology, Hospital Infanta Leonor, Madrid, Spain.
Pediatric Dermatology
|November 27, 2014
Summary
Dermatofibrosarcoma protuberans (DFSP) is rare in children, with an estimated annual incidence of 1.02 cases per million. Surgical excision is effective, with low recurrence and no metastasis observed in this pediatric study.
Area of Science:
- Pediatric Oncology
- Dermatopathology
- Epidemiology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma.
- Limited data exists on the incidence and management of pediatric DFSP.
Purpose of the Study:
- To estimate the annual incidence of DFSP in children (<18 years).
- To review the management and outcomes of pediatric DFSP cases.
Main Methods:
- Retrospective review of pediatric DFSP patients (2000-2011) in Madrid, Spain.
- Analysis of incidence, tumor characteristics, treatment, and follow-up data.
Main Results:
- Average annual incidence: 1.02 cases per million person-years.
- Median tumor size: 3.5 cm x 3 cm; median diagnostic delay: 36 months.
- Conventional, pigmented, and myxoid variants observed; surgical excision effective with one recurrence and no metastases.
Conclusions:
- DFSP incidence in children is low but significant.
- Surgical excision is a successful treatment modality for pediatric DFSP.
- Early diagnosis and complete surgical removal are crucial for optimal outcomes.
