Moll R, Lee I, Gould V E, Berndt R, Roessner A, Franke W W
Am J Pathol. 1987 May;127(2):288-304.
Examples of classical Ewing's tumors ("Ewing's sarcomas") of both skeletal and extraskeletal locations were analyzed for the expression of intermediate filament (IF) and cell junction proteins, with the use of immunofluorescence and immunoelectron microscopy as well as gel electrophoresis. In all 11 tumors examined vimentin filaments were abundant. A type of plaque-bearing small cell junction, which is common in these tumors but difficult to classify by morphologic criteria, was identified by antibodies to desmoplakins as true desmosomes. These were found in all cases, although in a very variable proportion of cells. Some of these junctions were associated with vimentin IFs. In addition, 9 of the cases examined showed scattered or clustered cells expressing the simple-epithelium type cytokeratins 8 and 18. Moreover, 3 cases displayed dispersed or clustered cells producing neurofilaments. The value of these observations, notably the cell type heterogeneity, for the diagnosis of tumors of this group is discussed. The results further indicate that Ewing's tumors are derived from a primitive, pluripotential cell that may differentiate, in variable proportions, into cells with mesenchymal, epithelial, and, more rarely, even neural features, suggesting that this tumor should be regarded as a blastoma, rather than as a true sarcoma.
利用免疫荧光、免疫电子显微镜以及凝胶电泳技术,对骨骼和骨骼外部位的经典尤因肿瘤(“尤因肉瘤”)样本进行了中间丝(IF)和细胞连接蛋白表达情况的分析。在所检测的全部11个肿瘤中,波形蛋白丝丰富。一种带有斑块的小细胞连接结构,在这些肿瘤中很常见,但通过形态学标准难以分类,利用桥粒斑蛋白抗体鉴定其为真正的桥粒。所有病例均发现有桥粒,不过在细胞中的比例差异很大。其中一些连接结构与波形蛋白中间丝相关。此外,在所检测的病例中,有9例显示表达简单上皮型细胞角蛋白8和18的细胞呈散在或聚集分布。而且,有3例显示产生神经丝的细胞呈分散或聚集分布。讨论了这些观察结果,尤其是细胞类型异质性,对于该组肿瘤诊断的价值。结果进一步表明,尤因肿瘤起源于一种原始的多能细胞,这种细胞可能以不同比例分化为具有间充质、上皮特征的细胞,甚至更罕见地分化为具有神经特征的细胞,这表明该肿瘤应被视为母细胞瘤,而非真正的肉瘤。