软组织外周性原始神经外胚层瘤临床病理及免疫组化分析

目的:分析软组织外周性原始神经外胚层瘤(pPNET)临床病理及免疫组化特征,为该肿瘤的诊断与治疗提供依据。方法:对8例pPNET组织常规处理,瑞氏-苏木素(HE)、过碘酸Schifff反应(PAS)、网织纤维及免疫组织化学染色。结果:临床上大部分为青年人,四肢软组织肿块,生存期短,预后差,2年生存率为33%,5年生存率为0。组织学上小圆、卵圆或梭形瘤细胞被纤维组织分割成小叶,可有或无菊形团。PAS染色阴性,网染细胞间网状纤维少或无,免疫组化染色瘤细胞表达2个以上神经性标记。结论:软组织pPNET为一种高度恶性的神经上皮瘤,临床病理与骨外Ewing瘤、Askin瘤比较无显著性意义。

CLINICAL PATHOLOGY AND IMMUNHISTOCHEMISTRY OF PERIPHERAL PRIMITIVE NEUROECTODERNAO TUMOR IN SOFT TISSUE

Objective:To study the features of both clinical pathology and immunohistochem-istry of peripheral primi tive neuroectodermal tumor(pPNET)for the diagnosis and treatment of the tu mor.Method:Eight pPNET samples were treated with general paraffin section,followed by HE,PAS,reticu lar fiber and immunohistochemistry stainings.Results:The small round-to-oval or shuttle shape tumor cells in histology were surrounded and cut apart the lobular structure by fibrous tissue,which had Homer-Wright structure or not.A few tumor cells were negative by PAS and there were no or few reticular fibers among the tumor cells.The tumor cells were expressed two nervous marks at least by immunohisto chemistry staining.Conclusion:pPNET of the soft tissue is one kind of higher malig nant neuroepithelioma and it has no significantly difference from Ewing's tumor located outside the bone and Askin's tumor.