CAJ | 학술논문

目的：探讨原发性肾脏血管母细胞瘤的临床表现、影像特征、病理及免疫组化特点并文献复习。方法收集整理2011～2013年我科收治的3例原发性肾脏血管母细胞瘤病例资料，并检索复习相关文献。结果例1为40岁男性，因右侧腰部不适检查发现右肾占位；例2为50岁男性，因体检发现左肾占位；例3为60岁女性，因左输尿管结石检查发现右侧脾肾间隙占位。3例患者均行腹腔镜下保留肾单位手术，术后病理为“肾血管母细胞瘤”。文献回顾中，国内外原发性肾脏血管母细胞瘤共15例报道，平均年龄49岁，肿瘤平均大小：4．5（1．2～9）cm。12例行肾根治切除，3例行肾部分切除。平均随访时间20.5（2～108）个月，均无转移复发报道。免疫组织化学结果示18例均有S‐100阳性表达，16例为神经元特异性烯醇化酶（NSE）、抑制素α（α‐in‐hibin）阳性，其中14例波形蛋白（vimentin）阳性（78％），7例Ki67（＜1％）。结论原发性肾脏血管母细胞瘤影像学表现与肾透明细胞癌及乏脂肪肾血管平滑肌脂肪瘤较难鉴别，病理及免疫组织化学特殊。近年来行肾部分切除术比例增加，目前无转移复发报道。
목적：탐토원발성신장혈관모세포류적림상표현、영상특정、병리급면역조화특점병문헌복습。방법수집정리2011～2013년아과수치적3례원발성신장혈관모세포류병례자료，병검색복습상관문헌。결과례1위40세남성，인우측요부불괄검사발현우신점위；례2위50세남성，인체검발현좌신점위；례3위60세녀성，인좌수뇨관결석검사발현우측비신간극점위。3례환자균행복강경하보류신단위수술，술후병리위“신혈관모세포류”。문헌회고중，국내외원발성신장혈관모세포류공15례보도，평균년령49세，종류평균대소：4．5（1．2～9）cm。12례행신근치절제，3례행신부분절제。평균수방시간20.5（2～108）개월，균무전이복발보도。면역조직화학결과시18례균유S‐100양성표체，16례위신경원특이성희순화매（NSE）、억제소α（α‐in‐hibin）양성，기중14례파형단백（vimentin）양성（78％），7례Ki67（＜1％）。결론원발성신장혈관모세포류영상학표현여신투명세포암급핍지방신혈관평활기지방류교난감별，병리급면역조직화학특수。근년래행신부분절제술비례증가，목전무전이복발보도。
ABSTRACT:Objective To discuss the clinicopathological and imaging characteristics of primary renal haemangioblastoma and review the literature .Methods Clinical data of 3 cases of primary renal haemangioblastoma treated during 2011 to 2013 were reviewed ,and related literature was retrieved and discussed .Results Case 1 was a 40‐year‐old male who found a solid mass on the right kidney during a checkup for low back pain .Case 2 was a 50‐year‐old male who found a solid mass on the left kidney during a routine examination .Case 3 was a 60‐year‐old female with ureteral calculus on opposite sides .Laparoscopic nephron sparing surgery was performed .Literature reviewed obtained 15 reports on primary renal haemangioblastoma .The av‐erage patients'age was 49 years ,and the mean diameter of tumors was 4 .5 cm (1 .2 -9 )cm .Radical nephrectomy was per‐formed on 12 cases and nephron sparing surgery was performed on 3 cases .During the average follow‐up of 20 .5 months (2‐108)m ,no recurrence or metastasis was reported .Neuronspecific enolase (NSE) ,a‐inhibin and S100 were positive in most tumor cells .Vimentin was positive in 14 cases (78% ) and Ki67 was positive in 7 cases .Conclusions The CT characteristics of renal haemangioblastoma are hard to differentiate from other renal diseases ,such as renal clear cell carcinoma and renal an‐giomyolipoma ,which can be distinguished by immunohistochemistry .Nephron sparing surgery is preferred in recent years and there has been no report on metastasis and recurrence so far .