CAJ | 학술논문

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。
목적：탐토인동신세포암(renal cell carcinoma，RCC)적림상병리특정、분류、진단여감별진단。방법：수집2003년~지금호남성인동의원3례인동RCC병례，기중남성2례，녀성1례，년령5.5~9세。진행광경급면역조화검측중신분류。결과：1례경하이유두상결구배렬포장투량적암세포위주，유두간가견섬유、혈관급염세포침윤，반유교다개화소체결구；기여2례경하균이실성소색상、선관상배포적기산성과립암세포위주，조성구역유소량투명암세포배렬성불전형유두상결구，미견개화소체；면역조화결과：기중1례표체TFE3、Vimentin、CK-pan화CEA；제2례표체Vimentin、CK-pan、CEA급p53；제3례표체Vimentin、CK-pan、CEA、NSE、CgA、Syn급Ki-67。결론：인동RCC교소견，HE형태하이유두상결구배렬적투명암세포류형수결합TFE3면역조직화학혹기인검측등수단명학진단。술전채용정맥화료능제고종류완정절술솔。인동RCC정체예후여성인상비교호，단Xp11.2역위/TFE3기인융합상관성신암(Xp11 RCC)예후교투명세포성신세포암(clear cell renal cell carcinoma，CCRCC)차，유우기재인동기다표현위타성진전，수장기적수방관찰。
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.