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目的：探讨原始神经外胚层瘤的诊断标准及鉴别诊断。方法：病理形态观察及免疫组化标记。结果：该肿瘤形态特征可见到纤维型菊形团和明显纤维组织分隔成小叶，瘤细胞内糖原很少，能表达2种以上神经性标记。结论：骨和软组织的原始神经外胚层瘤的诊断，必须进行免疫组化标记，尤其是神经性标记和粘液染色，在排除其它小细胞性肿瘤的基础上才能确诊。
Objective: To investigate the diagnostic criteria and differential diagnosis of primitive neuroectodermal tumors. Methods: Pathological observations and immunohistochemical markers. RESULTS: The morphological characteristics of the tumor showed that the fiber-shaped chrysanthemum-shaped group and the distinct fibrous tissue were separated into lobulars. There were few glycogens in the tumor cells and more than two types of neurogenic markers could be expressed. Conclusion: The diagnosis of primitive neuroectodermal tumors of bone and soft tissue must be performed with immunohistochemical markers, especially neurological markers and mucin staining, which can be diagnosed based on the exclusion of other small cell tumors.