Pediatric Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor) - A Case Report -
엄대운, 강길현, 이기원, 최수정,
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엄대운 ( Eom Dae-Woon )
울산대학교 의과대학 강릉아산병원 병리학교실
강길현 ( Kang Gil-Hyun )
울산대학교 의과대학 강릉아산병원 진단병리과
이기원 ( Lee Gi-Won )
울산대학교 의과대학 강릉아산병원 정형외과학교실
최수정 ( Choi Soo-Jung )
울산대학교 의과대학 강릉아산병원 방사선과학교실
KMID : 0357920070410030183
Abstract
A Bednar tumor is a rare neoplasm of intermediate malignant potential that accounts for 1-5% of all cases of dermatofibrosarcoma protuberans (DFSP). This tumor is considered a pigmented variant of DFSP, because the clinical and histological findings resemble DFSP. The diagnosis is commonly made in early to middle adult life except in cases with melanin containing cells. In the case presented here, the patient was a 3-year-old male who presented with a painless slow-growing 2.0×1.5×1.0 cm mass on the dorsal aspect of his right hand. Histological examination of the biopsy specimen revealed typical features of a Bednar tumor, which was composed of CD34 positive monomorphous spindle shaped cells arranged in a storiform fashion with moderate mitotic activity (up to 5 per 10 HPF) and scattered pigmented cells with dendritic processes. We report a rare case of Bednar tumor affecting a pediatric patient and review the medical literatures.
키워드
Dermatofibrosarcoma;Pigmentation;Pediatrics
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