Oda H, Shiga J, Machinami R
Department of Pathology, Faculty of Medicine, University of Tokyo, Japan.
Cancer. 1993 Apr 1;71(7):2286-91. doi: 10.1002/1097-0142(19930401)71:7<2286::aid-cncr2820710719>3.0.co;2-3.
Clear cell sarcoma of kidney (CCSK) is an aggressive renal tumor occurring most frequently in the second and third years of life. Although it has been reported in the literature in patients as old as 21 years of age, there has been no report focusing on adults with CCSK. The authors describe two cases of CCSK in men and the findings at autopsy.
Histologically, the renal tumors consisted of a homogeneous proliferation of polygonal or short spindle cells with faintly eosinophilic cytoplasm supported by a network of small vessels. Ultrastructurally, clusters of tumor cells were surrounded by collagen bundles. Elongated cytoplasmic processes, dilated rough endoplasmic reticulum, and intermediate filaments in the cytoplasm; primitive desmosome-like junctional structures; and incomplete basal lamina were observed. Immunohistochemically, tumor cells had positive results only for vimentin. Cytokeratins, epithelial membrane antigen, desmin, myoglobin, S-100 protein, neuron-specific enolase, and chromogranin A were not detected in the tumor cells. Both patients died within 2 years of onset of disease, and autopsy showed widespread metastases, especially in the soft tissue and bone.
Morphologic findings and an aggressive clinical course with metastasis to the bone and soft tissue are supportive of the diagnosis of CCSK in these patients.
肾透明细胞肉瘤（CCSK）是一种侵袭性肾肿瘤，最常发生于儿童2至3岁时。尽管文献报道过年龄高达21岁的患者患有该病，但尚无针对成年CCSK患者的报道。作者描述了两例男性CCSK病例及尸检结果。
组织学上，肾肿瘤由多边形或短梭形细胞均匀增生构成，细胞质呈淡嗜酸性，有小血管网络支持。超微结构上，肿瘤细胞簇被胶原束包围。观察到细胞质中有伸长的胞质突起、扩张的粗面内质网和中间丝；原始的桥粒样连接结构；以及不完整的基膜。免疫组化方面，肿瘤细胞仅波形蛋白呈阳性。肿瘤细胞中未检测到细胞角蛋白、上皮膜抗原、结蛋白、肌红蛋白、S-100蛋白、神经元特异性烯醇化酶和嗜铬粒蛋白A。两名患者均在疾病发作后2年内死亡，尸检显示有广泛转移，尤其是在软组织和骨骼。
形态学表现以及伴有骨和软组织转移的侵袭性临床病程支持这些患者为CCSK的诊断。
