Journal of Pathology and Translational Medicine

Progression of Dermatofibrosarcoma Proruberans to Malignant Fibrous Histiocytoma: Report of a case.: Hye Rim Park, Eun Ha Jung, Jin Hee Sohn; Korean J Pathol. 1997;31(12):1324-1327.

Abstract PDF: A 52-year-old woman was admitted with a palpable mass on the abdominal wall. This tumor had developed at the same site of a previously resected dermatofibrosarcoma protuberans. The biopsy specimen from the recurrent tumor revealed malignant fibrous histiocytoma. Immunohistochemistry with anti-CD34 antibody revealed diffuse linear positivity on the tumor cells of dermatofibrosarcoma protuberans and negativity on malignant fibrous histiocytoma. Anti CD34 antibody was valuable for a differential diagnosis of these two lesions.

Pediatric Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor): A Case Report.: Dae Woon Eom, Gil Hyun Kang, Key Won Lee, Soo Jung Choi; Korean J Pathol. 2007;41(3):183-186.

Abstract PDF: 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.

Dermatofibrosarcoma Protuberans of the Parotid Gland: A Case Report.: Ok Jun Lee, David Y Pi, Daniel H Jo, Kyung Ja Cho, Sang Yoon Kim, Jae Y Ro; Korean J Pathol. 2004;38(4):276-279.

Abstract PDF: Dermatofibrosarcoma protuberans (DFSP) typically presents during the early or mid-adult life, and the most common site of origin is the skin on the trunk and proximal extremities. DFSP of the parotid gland is extremely rare and only one case has been reported in the literature. We present here a case of a 30-year-old woman with DFSP occurring in the parotid gland, and we discuss the differential diagnosis. The patient is alive and doing well one year after her operation.

Bednar Tumor: A case report with discusion of histogenesis.: Mee Soo Chang, Kye Yong Song; Korean J Pathol. 1993;27(6):659-662.

Abstract PDF: The authors examined a case of Bednar tumor(pigmented dermatofibrosarcoma protuberans). A 35-year-old woman presented with protruding black back mass 3x2.5 cm in surface dimension and 1 cm in heigh, which has grown slowly for 30 years. Microscopically the lesion is characterized by spindle cells arranged in storiform pattern and admixed with melanin containing dendritic cells. Ultrastructural study revealed three cell populations: (1) cells resembling fibroblasts, (2) cells resembling perineural cells, and (3) melanocytes. The immunohistochemical study using S-100 protein alpha-1 antitrypsin, antichymotrypsin and EMA revealed negative reaction in spindle fibroblastic tumor cells. The histogenesis of this neoplasm remains controversial; neuroectoderm, perineural cell, fibroblast and histiocyte. Through the ultrastructural study and immunohistochemical study, the authors suggested that Bedner tumor arises form the neuroectoderm with bidirectional to perineural and fibroblastic differentiation.

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