Sokolova I N, Lebedev V I
Arkh Patol. 1984;46(6):33-40.
Nine post-mortem cases included 5 boys and 4 girls at the age from 1 1/2 to 13 years. The ratio right/left kidney was 6 to 3; average tumour weight 780 g, higher than in typical nephroblastoma. Bone metastases were found in 6 cases, the therapy was inefficient. Light-microscopic examination revealed structural polymorphism (typical for nephroblastoma areas, fibroses, hyalinosis, myxomatosis), smooth muscles in 2 cases. Electron microscopy of clear cells revealed fibrillar component developed at a various degree, vacuoles, pynocytosis vesicles, large nuclei with fine structure of chromatin. The results indicate the clear-cell variant of nephroblastoma is a sarcomatoid tumour with characteristic structure and uncommon clinical behaviour which makes necessary a special diagnostic and therapeutical approach.
9例尸检病例包括5名男孩和4名女孩,年龄在1.5岁至13岁之间。左右肾比例为6比3;肿瘤平均重量780克,高于典型肾母细胞瘤。6例发现骨转移,治疗无效。光镜检查显示结构多态性(肾母细胞瘤典型区域、纤维化、玻璃样变、黏液瘤样变),2例有平滑肌。透明细胞的电镜检查显示纤维成分有不同程度发育、空泡、胞饮小泡、染色质结构精细的大细胞核。结果表明,肾母细胞瘤的透明细胞变体是一种具有特征性结构和罕见临床行为的肉瘤样肿瘤,这使得有必要采取特殊的诊断和治疗方法。