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A Case of Congenital Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor) in a Patient with Fanconi Anemia


Address correspondence to Mi-Woo Lee, M.D., Ph.D, Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnapdong Songpagu, Seoul 138-736, Korea, or e-mail:


Abstract:  Bednar tumor is a rare pigmented variation of dermatofibrosarcoma protuberans, present in 1 to 5% of all patients with dermatofibrosarcoma protuberans. No significant clinicopathologic differences exist between Bednar tumor and conventional dermatofibrosarcoma protuberans apart from the presence of scattered nonneoplastic pigmented dendritic cells in the former. Although most dermatofibrosarcoma protuberans occur in adults, they may be rarely present at birth. Fanconi anemia is a genetically heterogeneous chromosomal instability syndrome, characterized by multiple congenital anomalies, progressive bone marrow failure, and a predisposition to malignancy. We describe here a patient with Fanconi anemia who had a congenital Bednar tumor. To our knowledge, this is the first such patient described with both dermatofibrosarcoma protuberans and Fanconi anemia.

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