Schmidt D, Harms D
Abt. Paidopathologie der Christian-Albrechts-Universität zu Kiel.
Klin Padiatr. 1990 Jul-Aug;202(4):224-9. doi: 10.1055/s-2007-1025524.
The diagnosis and differential diagnosis of malignant soft tissue tumors not infrequently poses great difficulties, especially in those cases which lack any feature of differentiation by conventional light microscopy. These difficulties have been partially resolved through the application of ultrastructural investigations. Recently considerable progress has been achieved using immunohistochemistry. At the Kiel Pediatric Tumor Registry we were able to reduce the percentage of unclassified soft tissue sarcomas from 17.6% in 1982 to 4.5% in 1989. Particularly useful were antibodies against the different types of intermediate filaments, muscle-specific actin, myoglobin, and the neural markers neuron-specific enolase and protein S-100. In contrast to the expected immunophenotype rhabdomyosarcomas, malignant peripheral neuroectodermal tumors and malignant schwannomas showed expression of cytokeratins. Moreover, in many cases rhabdomyosarcomas and synovial sarcomas expressed neural markers. Ewing's sarcoma and malignant peripheral neuroectodermal tumor are histogenetically related, but differ in grade of neural differentiation. In all soft tissue sarcomas immunohistochemistry is very useful to obtain information on the cellular heterogeneity. Despite the great achievements not every soft tissue sarcoma can be diagnosed with certainty. There will always be a baseline of unclassified cases due to problems which are not caused by the tumor itself but rather by diagnostic circumstances.
恶性软组织肿瘤的诊断及鉴别诊断常常极具难度,尤其是那些在传统光学显微镜下缺乏任何分化特征的病例。通过应用超微结构研究,这些难题已得到部分解决。近来,免疫组织化学取得了显著进展。在基尔儿科肿瘤登记处,我们成功地将未分类软组织肉瘤的比例从1982年的17.6%降至1989年的4.5%。针对不同类型中间丝、肌肉特异性肌动蛋白、肌红蛋白以及神经标志物神经元特异性烯醇化酶和蛋白质S - 100的抗体尤为有用。与预期的免疫表型相反,横纹肌肉瘤、恶性外周神经外胚层肿瘤和恶性神经鞘瘤显示出细胞角蛋白的表达。此外,在许多病例中,横纹肌肉瘤和滑膜肉瘤表达神经标志物。尤因肉瘤和恶性外周神经外胚层肿瘤在组织发生学上相关,但神经分化程度不同。在所有软组织肉瘤中,免疫组织化学对于获取细胞异质性的信息非常有用。尽管取得了巨大成就,但并非每例软组织肉瘤都能被明确诊断。由于并非由肿瘤本身而是由诊断情况所导致的问题,总会存在一定比例的未分类病例。