Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans
Autor: | Duncan MacGregor, Yew Loong Loo, Christopher J. Coombs, Daniel J Reilly, William Alexander, Daniel J. Wilks |
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Rok vydání: | 2021 |
Předmět: |
Male
Retrospective review medicine.medical_specialty Skin Neoplasms business.industry Incidence (epidemiology) General surgery Dermatofibrosarcoma Soft tissue Margins of Excision Mean age Fibrohistiocytic tumor medicine.disease Dermatofibrosarcoma protuberans medicine Humans Surgery Surgical excision Single institution Neoplasm Recurrence Local business Child Follow-Up Studies Retrospective Studies |
Zdroj: | Annals of plastic surgery. 88(3) |
ISSN: | 1536-3708 |
Popis: | 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. |
Databáze: | OpenAIRE |
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