CAJ | 학술논문

目的探讨散发性肾血管母细胞瘤的临床病理特征、免疫表型、诊断及鉴别诊断.方法对2010和2013年收集的2例散发性肾血管母细胞瘤患者临床资料、组织学、免疫组织化学(EliVision法)等进行观察分析,并复习相关文献.结果例1为40岁男性,例2为45岁女性,体检发现右肾占位.大体均为右肾中极外突性肿物,切面灰红灰褐色,边界清楚.镜下由两种成分构成:即不同成熟阶段的毛细血管和毛细血管网之间丰富的间质细胞.例1为细胞亚型,以间质细胞为主；例2为网状亚型,以毛细血管网为主.免疫组织化学显示间质细胞波形蛋白、神经元特异性烯醇化酶、S-100蛋白及α抑制素均为阳性,CK以及HMB45、Melan A、CD56、突触素、上皮细胞膜抗原均为阴性,Ki-67阳性指数＜1％,CD34血管壁阳性.结论散发性肾血管母细胞瘤是肾原发性良性间叶性肿瘤,临床罕见,诊断主要依赖特征性的形态学表现,并通过免疫组织化学与临床检查除外其他疾病.
목적 탐토산발성신혈관모세포류적림상병리특정、면역표형、진단급감별진단.방법 대2010화2013년수집적2례산발성신혈관모세포류환자림상자료、조직학、면역조직화학(EliVision법)등진행관찰분석,병복습상관문헌.결과 례1위40세남성,례2위45세녀성,체검발현우신점위.대체균위우신중겁외돌성종물,절면회홍회갈색,변계청초.경하유량충성분구성:즉불동성숙계단적모세혈관화모세혈관망지간봉부적간질세포.례1위세포아형,이간질세포위주；례2위망상아형,이모세혈관망위주.면역조직화학현시간질세포파형단백、신경원특이성희순화매、S-100단백급α억제소균위양성,CK이급HMB45、Melan A、CD56、돌촉소、상피세포막항원균위음성,Ki-67양성지수＜1％,CD34혈관벽양성.결론 산발성신혈관모세포류시신원발성량성간협성종류,림상한견,진단주요의뢰특정성적형태학표현,병통과면역조직화학여림상검사제외기타질병.
Objective To investigate the clinicopathological characteristics of sporadic renal hemangioblastoma.Methods Two cases of sporadic renal hemangioblastoma were studied by histological and immunohistochemical staining,along with review of the literature.Results Both cases were presented with a renal mass during annual physical examination with an age of 40 and 45 years,respectively.The lesions located in the middle portion of the kidney with a sharp tumor border.Histopathologically,the tumors were characterized by two major components:capillary and stromal cells.Immunohistochemically,the stromal cells were positive for vimentin,NSE,S-100 protein and inhibin-α,and negative for CK,HMB45,Melan A,EMA,CD56 and syn.Both tumors showed a very low level of Ki-67 labeling.The endothelial cells were positive for CD34.Conclusion Sporadic renal hemangioblastoma is a rare benign neoplasm,the diagnosis of which is based on the characteristic follicular architecture and an exclusion of other carcinomas of the kidney.