Congenital Dermatofibrosarcoma Protuberans: A Case Report

다국어 초록 (Multilingual Abstract)

Purpose: Dermatofibrosarcoma protuberans is a rare tumor, accounting for 0.1% of all malignant tumors. Although metastasis is very uncommon, local recurrence occur frequently. Dermatofibrosarcoma protuberans occurring in children is even more rare; this is the first case report of congenital dermatofibrosarcoma protuberans in Korea. Methods: The patient is a 14-month-old male infant with a lesion that was first thought to be a birthmark. The lesion grew larger, and a punch biopsy revealed dermatofibrosarcoma protuberans. A wide local excision was performed with a 2 cm peripheral resection margin beyond the gross tumor lesion. Deep fascia and a portion of muscle underneath the central part of the lesion were also taken. The surgical defect was covered by a split-thickness skin graft. Results: There has been no clinical sign of recurrence over one year after the surgery. Conclusion: A patient with congenital dermatofibrosarcoma protuberans detected at an early stage underwent a wide local excision of the tumor after accurate diagnosis was carried out by biopsy and immunohistochemical studies. There was no clinical evidence of tumor recurrence during over a 1-year follow-up.

참고문헌 (Reference)

1 Seong JY, "Plaque stage of dermatofibrosarcoma protuberans" 39 : 937-, 2001

2 Reddy C, "Dermatofibrosrcoma protuberans in children" 62 : 819-, 2009

3 Weinstein JM, "Congenital dermatofibrosarcoma protuberans: variability in presentation" 139 : 207-, 2003

4 Ng A, "Chemosensitivity in pediatric dermatofibrosarcoma protuberans" 27 : 100-, 2005

5 Patrizi A, "A pediatric case of dermatofibrosarcoma protuberans: an immunohistochemical study" 17 : 29-, 2000

6 Choi YW, "A case of dermatofibrosarcoma protuberans treated with Mohs micrographic surgery and purse-string suture repair" 33 : 1108-, 1995

7 Jung JH, "A case of congenital dermatofibrosarcoma protuberans with a histologic feature of bednar tumor" 39 : 5-, 2001

8 Yoon YM, "A case of childhood dermatofibrosarcoma protuberans" 39 : 609-, 2001