摘要
节细胞神经母细胞瘤(ganglioneuroblastoma,GNB)与神经母细胞瘤(meuroblastoma,NB)和神经节细胞瘤(gan-glioneuroma,GN)是同源性不同分化程度的肿瘤。前者的分化程度界于后两者之间。本文报告我院40年来11例GNB的研究结果,探讨这种可见肿瘤的临床病理学特征、免疫组化及癌基因产物表达情况。10例患者为2~6岁,1例为44岁;8例男性,3例女性;原发部位7例在腹膜后,4例在纵隔。组织学分型,依据肿瘤内成热节细胞与神经母细胞的比例和分布不同,参照shimada分类法分为中间混合型(包括A型3例、B型2例)和结节型6例。免疫组化染色结果为NSE9/9、CGA8/11S-1009/11、P53蛋白3/11、C—erb—B29/11、P21蛋白5/6。NSE、CGA和S—100可作为GNB的标记物,具体应用时应2~3项合用并结合组织学图像,以提高诊断正确率。GNB内肿瘤基因产物的表达提示其发生与这些肿瘤基因有关,但未发现它们之间有协同现象。说明该肿瘤的发生机理十分复杂,可能是多环节调节异常的结果。对该肿瘤的深入研究有待于资料的进一步积累。
cases of the ganglioneuroblastoma were studies. Ages of 10 cases ranged from 2 to 6 years old. Only one case was 44 years old. There were 8 males and 3 females. Teh primary tumor sites were retroperitoneum (7 cases )and mediastinum (4 cases). Histologiocally, the tumors were devided on the basis of the morphologic features of neuroblastic elements into three subgroups:' Type A Intermixed”, having individual microscopic nests of neuroblasts(n=3);' Type B Intermixed”, having grossly visible nodule(s) of neuroblastic proliferation(n=6). Immunohistochemical studies showed 9/g cases ositive for NSE, 8/11 for CGA ang 9/11 for S-100. These results suggested that NSE, CGA and S-100 can be used as the markers of thetmuor. The studies also showed 3/11 cases positive for P 53 protein, 9/11 for C-erb-B2 and 5/6 for P 21 protein, indicating somerelationships bet ween these tumor genes and the proliferation of thistumor.
关键词
神经母细胞瘤
病理学
免疫组织化学
Ganglioneuroblastoma Neuroendocrine oncogene Immunohistochemistry