Choi Y J, Jung W H, Jung S H, Park C
Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 1994 Sep;35(3):336-43. doi: 10.3349/ymj.1994.35.3.336.
Immunohistochemical study and flow cytometric DNA analysis were done on seven cases of clear cell sarcoma of the kidney (CCSK) to speculate its histogenesis and to access the diagnostic usefulness of these methods in the differential diagnosis of Wilm's tumor. Clinically, CCSK is a rare malignant renal tumor of children with a propensity to metastasize to bone. Arborizing vascular pattern surrounding the tumor cells which have clear cytoplasm is characteristic histologic finding. Immunohistochemically, only vimentin was diffusely demonstrated in the tumor cell membrane and cytoplasm. In flow cytometric DNA analysis, four cases showed diploidy and two cases near diploidy. CCSK is a separate disease entity with characteristic clinicopathologic, immunohistochemical and flow cytometric findings in distinction from Wilms' tumor. Considering the histologic and immunohistochemical findings, the possible histogenetic mechanism of CCSK seems to be in common with congenital mesoblastic nephroma (CMN), that is primitive mesenchymal cells which committed early stromagenic activity.
对7例肾透明细胞肉瘤（CCSK）进行了免疫组织化学研究和流式细胞术DNA分析，以推测其组织发生，并评估这些方法在肾母细胞瘤鉴别诊断中的诊断价值。临床上，CCSK是一种罕见的儿童恶性肾肿瘤，易转移至骨。肿瘤细胞周围呈树枝状的血管模式以及肿瘤细胞胞质清晰是其特征性组织学表现。免疫组织化学方面，仅波形蛋白在肿瘤细胞膜和细胞质中呈弥漫性表达。在流式细胞术DNA分析中，4例显示二倍体，2例接近二倍体。CCSK是一种独立的疾病实体，具有与肾母细胞瘤不同的特征性临床病理、免疫组织化学和流式细胞术表现。考虑到组织学和免疫组织化学结果，CCSK可能的组织发生机制似乎与先天性中胚层肾瘤（CMN）相同，即早期具有基质生成活性的原始间充质细胞。
