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外周原始神经外胚瘤的临床病理分析及免疫组化观察

 左大镒 肖宝芝 戈立东 朱德为

摘 要
  【目的】探讨原始神经外胚瘤(PNET)与其它恶性小细胞肿瘤的鉴别要点。【方法】应用免疫组化SP法对14例PNET进行免疫组化标记,同时结合临床病理资料进行分析。【结果】14例PNET中,除胸壁3例外,其余的均沿身体纵轴分布,为一界限性肿块,瘤细胞密集呈巢状排列,常被致密纤维组织分隔成分叶状或腺泡状,可见神经性玫瑰花形物形成;免疫组化神经性标记至少2项以上阳性。【结论】临床病理学观察结合免疫组化,在PNET与其它恶性小细胞肿瘤的鉴别诊断中有重要意义。
关键词 肿瘤,胚胎性和混合性; 病理学,临床; 免疫组织化学

Clinicopathologic Analysis and Immunohistochemical Observation
of Peripheral Primitive Neuroectodermal Tumor

ZUO Dayi, XIAO Baozhi, GE Lidong,et al
The 169th Central Hospital of PLA,Hunan Province,Hengyang,421002

Abstract
  【Objective】To investigate the differential diagnosis between primitive neuroectodermal tumor(PNET) and other malignant small cell tumors.【Methods】In combination with analysis of clinicopathological data,paraffin embedded biopsies from 14 patients with PNET were studied immunohistochemically.【Results】It was found that this tumor frequently developed along the longitudinal axis of the body excepting 3 cases in chest and formed localized mass.The neoplastic cells were arranged in nests,lobules or alveoli separated by dense fibrous tissues.Homer-Wright rosettes were often seen.Immunohistochemically PNET expressed at least more than two positive neural markers.【Conclusions】It is suggested that clinicopathological investigation in combination with immunohitochemical observation shows important significance in the differential diagnosis between PNET and other malignant small cell tumors.
Key word neoplasms,embronal and mixed; pathology,clinical; immunohistochemistry

  原始神经外胚瘤(Primitive Neuroectodermal Tumor,PNET)是一组可发于中枢神经系统,也可发生于周围神经系统的恶性小细胞肿瘤。该肿瘤由于分化程度差,多呈原始的未分化状况,常规切片诊断有一定困难,常需与其它类恶性小细胞肿瘤鉴别。本组报道14例PNET的临床病理分析及免疫组化观察,以期为临床鉴别诊断提供参考。

1 材料与方法
1.1 标本来源及处理 14例PNET来源于本院1982~1996年的活检标本8例,尸检1例,收集衡阳市疑难病理交流片病例5例。所有标本均为10%福尔马林固定,石蜡包埋,4 μm连续切片,作HE染色;6例作网状纤维染色;4例作PAS染色;3例作Masson三色染色;2例作冰冻切片脂肪染色。所有病例均用SP法(福州迈新公司提供的抗体)进行了NSE,NF,S-100蛋白,Synaptophysin,CHg-A,GF

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