Schmidt D, Harms D
Institut für Pathologie, Klinikum der Christian-Albrechts-Universität zu Kiel.
Klin Padiatr. 1988 May-Jun;200(3):236-42. doi: 10.1055/s-2008-1033715.
158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).
对基尔儿科肿瘤登记处记录的158例合作性尤因肉瘤试验（CESS 81/86）病例进行了传统光学显微镜检查和免疫组织化学研究。其中典型尤因肉瘤77例，70例位于骨骼，7例位于软组织。14例非典型尤因肉瘤中，骨和软组织各7例。与典型尤因肉瘤不同，非典型尤因肉瘤细胞更大，异染色质更多。典型和非典型尤因肉瘤波形蛋白染色均呈阳性，其他染色均为阴性，尤其是神经元特异性烯醇化酶（NSE）。55例诊断为恶性外周神经外胚层肿瘤（MPNT）。组织学上，这些肿瘤大多类似于非典型尤因肉瘤。免疫组织化学检查发现NSE、波形蛋白、蛋白质S-100、神经丝和胶质纤维酸性蛋白呈阳性反应。3例诊断为小细胞骨肉瘤。有2例骨未分化肉瘤、2例组织发生不明的软组织肉瘤和2例横纹肌肉瘤。根据Salzer-Kuntschik等人（1983年）建立的组织学消退分级分类，在可研究多药化疗反应的4例肿瘤中，分别有1例对应Ⅱ级和Ⅲ级，2例对应Ⅳ级。
