儿童肾细胞癌3例临床病理分析  

Pediatric renal cell carcinoma: clinicopathological analysis of 3 cases

作　　者：姜楠[1] 陈卫坚[1] 周峥珍[1] 刘敏[1] 张丽琼[1] 

机构地区：[1]湖南省儿童医院儿科研究所病理室,长沙410000

出　　处：《临床与病理杂志》2015年第5期787-793,共7页Journal of Clinical and Pathological Research

摘　　要：目的：探讨儿童肾细胞癌（renal cell carcinoma，RCC）的临床病理特征、分类、诊断与鉴别诊断。方法：收集2003年~至今湖南省儿童医院3例儿童RCC病例，其中男性2例，女性1例，年龄5.5~9岁。进行光镜及免疫组化检测重新分类。结果：1例镜下以乳头状结构排列胞浆透亮的癌细胞为主，乳头间可见纤维、血管及炎细胞浸润，伴有较多钙化小体结构；其余2例镜下均以实性巢索状、腺管状排布的嗜酸性颗粒癌细胞为主，灶性区域有少量透明癌细胞排列成不典型乳头状结构，未见钙化小体；免疫组化结果：其中1例表达TFE3、Vimentin、CK-pan和CEA；第2例表达Vimentin、CK-pan、CEA及p53；第3例表达Vimentin、CK-pan、CEA、NSE、CgA、Syn及Ki-67。结论：儿童RCC较少见，HE形态下以乳头状结构排列的透明癌细胞类型需结合TFE3免疫组织化学或基因检测等手段明确诊断。术前采用静脉化疗能提高肿瘤完整切术率。儿童RCC整体预后与成人相比较好，但Xp11.2易位/TFE3基因融合相关性肾癌（Xp11 RCC）预后较透明细胞性肾细胞癌（clear cell renal cell carcinoma，CCRCC）差，由于其在儿童期多表现为惰性进展，需长期的随访观察。Objective：To investigate the clinical pathological features, classification, differential diagnosis and treatment of Pediatric renal cell carcinoma. Methods：To valid our proposition, we reclassify children kidney carcinoma under both light microscope and immune histochemical detection. hTe samples we used were consisted of two male cases and one female case, whose age ranged from 5.5 to 9 years old. All of the three samples were collected from children＇s hospital of hunan province since 2003. Results：In our experiment, 1 case with papillary structures arranged in translucent cytoplasm of cancer cells mainly, meanwhile fibers, blood vessels and inflammatory cells infiltrated between nipple accompanied by more calcified bodies;The other two were solid nests or duct-like arrangement of the eosinophilic granular cancer cells based in, regional distribution of a small amount of focal atypical papillary structures, but no calciifed bodies in visible. Immunohistochemistry results：hTe expressions of case 1 were TFE3, Vimentin, CK-pan and CEA;case 2 expressed Vimentin, CK-pan, CEA and p53;case 3 expressed Vimentin, CK-pan, CEA, NSE, CgA, Syn and Ki-67. Conclusion：Renal cell carcinoma rarely happened to children, hTe HE form in clear cell types arranged in papillary structure should be combined with the TFE3 protein immunohistochemical and gene detection methods in diagnosis. Preoperative intravenous chemotherapy can improve the rate of complete tumor resection. The overall prognosis in children is better than adult, however renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions is poorer than clear cell renal cell carcinoma as to prognosis. In addition, since its performance is inert in childhood, better prognosis otfen requires long term observation.

关 键 词：儿童肾细胞癌 Xp11.2易位/TFE3基因融合相关性肾癌 透明细胞性肾细胞癌 

分 类 号：R737.11[医药卫生—肿瘤]