成人肾恶性横纹肌样瘤的研究:1例报告及文献复习

THE STUDY ON MALIGNANT RHABDOID TUMOR OF THE KIDNEY IN THE ADULT: A CASE REPORT AND REVIEW OF THE LITERATURE

应用组织化学、免疫组织化学及电镜技术对我国首例成人肾恶性横纹肌样瘤（ｍ ａｌｉｎｇａｎｔｒｈａｂｄｏｉｄｔｕｍ ｏｒ， ＭＲＴ） 进行了研究， 与婴幼儿ＭＲＴ相比本例有以下临床病理特点： （１） 以血尿为早期和唯一症状，肿块缺乏特征性ＣＴ 改变； （２） 光镜下大量ＰＡＳ阳性嗜酸性球形小体多位于细胞外， 电镜下细胞外小体由微丝构成并有界膜包绕； （３） 免疫组化显示肿瘤细胞向神经外胚层、横纹肌和组织细胞多向性分化； （４） 肿瘤侵袭力较低， 高度表达ｎｍ ２３－Ｈ１ 蛋白， 随访１８个月术后化疗效果佳， 提示其恶性度较婴幼儿ＭＲＴ 低。

The first case of adult malignant rhabdoid tumor (MRT) of kidney in China was studied using histochemical, immunohistochemical and electron microscopic techniques. Compared with MRT in infant, this case exhibited follwing clinicopathologic features: (1) The hemuresis was the only symptom in the early stage and the tumor mass did not present characteristic CT images; (2) Microscopically, there were a number of PAS positive eosinophilic globular bodies outside the cells. Ultrastructurally, the bodies outside of the cells were composed of microfilaments and surrounded by membrane; (3) Immunohistochemically, the tumor cells showed omniodirectional differentiation to neuroectoderm, rhabdmyocyte and histocyte; (4) The tumor showed lower invasiveness and over expression of nm23 H1 protein. The postoperative chemical therapy achieved success during 18 month follow up. It was suggested that the malignancy of MRT in this case was lower than that in infant.\;