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The Korean Journal of Pathology 2007;41(3): 183-186.
Pediatric Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor): A Case Report.
Dae Woon Eom, Gil Hyun Kang, Key Won Lee, Soo Jung Choi
1Departments of Pathology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea. edwjyh@gnah.co.kr
2Departments of Orthopedics, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.
3Departments of Radiology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.
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 2.0x1.5x1.0 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.
Key Words: Dermatofibrosarcoma; Pigmentation; Pediatrics