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Excellent survival of pediatric dermatofibrosarcoma protuberans in Taiwanese
Chun-Ru Chien1, Yih-Leong Chang, Dong-Tsamnn Lin
1National Taiwan University Hospital, Taipei, Taiwan.
Insights
Pediatric dermatofibrosarcoma protuberans (DFSP) in Taiwan shows excellent clinical outcomes. This study found high survival rates and minimal complications in young DFSP patients, highlighting effective treatment strategies.
Area of Science:
- Oncology
- Pediatric Oncology
- Dermatology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma.
- Pediatric DFSP is even rarer, with limited data on its clinical outcomes.
Purpose of the Study:
- To investigate the clinical outcomes of pediatric dermatofibrosarcoma protuberans (DFSP) in Taiwan.
- To evaluate the long-term prognosis and treatment effectiveness for young DFSP patients.
Main Methods:
- Retrospective analysis of pediatric DFSP patients treated between 1977 and 2002.
- Utilized institutional cancer registry and National Residency Registry for patient identification and outcome tracking.
- All patients underwent wide excision; no neoadjuvant or adjuvant therapies were administered.
Main Results:
- Twelve pediatric DFSP cases were confirmed pathologically.
- One instance of uncontrollable local recurrence and subsequent death was recorded.
- Achieved 10-year and 15-year overall survival rates of 100% and 83%, respectively, with minimal complications.
Conclusions:
- Pediatric DFSP in Taiwan demonstrates an excellent prognosis.
- Wide excision appears to be an effective primary treatment modality for pediatric DFSP.
- The findings support a favorable outlook for young patients diagnosed with DFSP in this region.
Abstract:
The purpose of this study was to explore the clinical outcome of pediatric dermatofibrosarcoma protuberans (DFSP) in Taiwan. Patients with DFSP diagnosed before 18 years and treated in our institute from 1977 to 2002 were identified through institutional cancer registry. After approved by institutional research ethic committee, active patient contact and linkage with National Residency Registry were done to explore their clinical outcome (Clinical Trials gov Identifier: NCT00173355). Twelve patients were identified. Pathological review confirmed DFSP for all of them. All of them underwent wide excision and none of them underwent neoadjuvant or adjuvant therapy. Nine patients underwent operation as initial therapy and three of them underwent salvage therapy. We recognized only one uncontrollable local recurrence and subsequent death. The 10-year and 15-year overall survival rates were 100 and 83%, respectively, without obvious complications. The prognosis of pediatric DFSP in Taiwanese was excellent.
