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KMID : 0363220160540050377
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Korean Journal of Dermatology
2016 Volume.54 No. 5 p.377 ~ p.380
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Congenital Dermatofibrosarcoma Protuberans Treated with Wide Excision
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Park Su-Kyung
Woo Su-Han
Kim Dae-Woo
Hwang Su-Ran
Park Jin
Yun Seok-Kweon
Kim Han-Uk
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Abstract
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Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor with intermediate malignancy that usually appears in adults. The congenital type is very rare, and only three cases have been reported so far in the Korean literature. A 2-month-old girl presented with a firm reddish-gray nodule on the middle of her back that had been present since birth. Histopathologically, the lesion was composed of fascicles of densely packed uniform spindle cells arranged in a storiform (mat-like) pattern. The cells had hyperchromatic nuclei and rare mitotic figures. On immunohistochemistry, the tumor cells were positive for CD34. Based on these findings, we made the diagnosis of congenital dermatofibrosarcoma protuberans. She underwent wide excision, and there was no evidence of recurrence during 24 months of follow-up. Our report is of interest because of the rarity of congenital dermatofibrosarcoma protuberans and its earliest detection reported in Korea.
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KEYWORD
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Congenital dermatofibrosarcoma protuberans, Wide excision
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