Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes
Marta Valdivielso-Ramos1, Antonio Torrelo, Minia Campos
1Department of Dermatology, Hospital Infanta Leonor, Madrid, Spain.
Insights
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.
Abstract:
Little is known about the incidence and management of dermatofibrosarcoma protuberans (DFSP) in children. We conducted a retrospective review of all patients younger than 18 years of age treated for DFSP over a period of 11 years (2000-2011) in Madrid, Spain. The sample consisted of 13 children. The average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases per million person-years (95% confidence interval 0.55, 1.73). Sites of involvement were diverse, with 15.3% of tumors found in acral locations. The median tumor size was 3.5 cm × 3 cm and the median time from apparent onset to diagnosis was 36 months. Histopathologic examination revealed conventional (77.0%), pigmented (15.4%), and myxoid (7.6%) variants. The mitotic index was consistently <5 per 10 high-power fields. All lesions were removed using surgical excision. One patient developed a local recurrence because of initial affected margins; none developed metastases. The median duration of clinical follow-up was 70.5 months. This study estimated the average annual incidence rate of DFSP in a population of patients younger than 18 years and reviewed the experience of several hospitals in the management of this tumor.
