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[目的]探讨外周原始神经外胚叶肿瘤(pPNET)的临床病理特征、诊断及鉴别诊断。[方法]应用常规病理检查和免疫组织化学EnVision方法,观察pPNET临床病理学特征。[结果]11例患者临床多以局部肿块首发,无特异症状。光镜下瘤组织主要由小圆形或卵圆形细胞组成,纤维组织分隔成结节状,可形成H-W菊形团,免疫组化染色CD99强阳性。[结论]外周原始神经外胚叶肿瘤的诊断依赖病理特征及免疫表型,并需与其他小细胞恶性肿瘤进行鉴别。
[Objective] To investigate the clinicopathologic features, diagnosis and differential diagnosis of peripheral primitive neuroectodermal tumor (pPNET). [Methods] The routine pathological examination and immunohistochemistry EnVision method were used to observe the clinicopathological features of pPNET. [Results] Most of the 11 patients were clinically starting with local mass and no specific symptoms. Under light microscope, the tumor mainly consisted of small round or oval cells, and the fibrous tissue was divided into nodules, which formed H-W chrysanthemum and strongly positive for CD99 by immunohistochemistry. [Conclusion] The diagnosis of peripheral primitive neuroectodermal tumors depends on the pathological features and immunophenotype, and should be differentiated from other small cell malignant tumors.