Onoda N, Tsutsumi Y, Kakudo K, Ozawa A, Niizuma K, Ohkido M, Osamura R Y
Department of Pathology, Tokai University School of Medicine, Isehara, Japan.
Acta Pathol Jpn. 1990 Dec;40(12):935-40.
An autopsy case of pigmented dermatofibrosarcoma protuberans (Bednár tumor) with systemic metastasis is reported. No previous example of this tumor showing widespread metastasis has been reported in the literature. The patient, a 45-year-old man, developed a tumor on the right upper arm. The tumor recurred twice and metastasized to other parts of the skin, lungs and brain during the 8-year clinical course. The primary tumor contained melanin-laden tumor cells and showed a storiform growth pattern. Autopsy confirmed multiple metastatic lesions in the skin, lungs, brain, thyroid, pancreas, stomach, small intestine and thigh muscles. The recurrent and metastatic tumors lacked both melanin production and the storiform arrangement, and instead revealed "fibro-sarcomatous" change with a herring-bone or interlacing pattern of growth.
报告了1例伴有全身转移的色素性隆突性皮肤纤维肉瘤（贝德纳瘤）尸检病例。文献中未报道过该肿瘤出现广泛转移的先例。患者为一名45岁男性，右上臂出现肿瘤。在8年的临床病程中，肿瘤复发两次，并转移至皮肤其他部位、肺和脑。原发肿瘤含有含黑色素的肿瘤细胞，呈车辐状生长模式。尸检证实皮肤、肺、脑、甲状腺、胰腺、胃、小肠和大腿肌肉中有多个转移病灶。复发和转移肿瘤既无黑色素生成，也无车辐状排列，而是呈现“纤维肉瘤样”改变，生长方式为鱼骨状或交错状。
