肾上皮样血管平滑肌脂肪瘤10例报道并文献复习  

Renal epithelioid angiomyolipoma:a report of 10 cases and literature review

作　　者：袁逸民 姜伟[1] 张胜捷[1] 张立进[1] 蒋昊[1] 陈蔚[1] 赵晓智[1] 郭宏骞[1] 

机构地区：[1]南京医科大学鼓楼临床医学院泌尿外科,江苏南京210008

出　　处：《现代泌尿外科杂志》2016年第3期214-217,共4页Journal of Modern Urology

摘　　要：目的探讨肾上皮样血管平滑肌脂肪瘤(EAML)的症状、诊断、影像学表现、病理特征、治疗方法及预后。方法通过对10例EAML患者临床资料的回顾性分析,结合相关文献,探讨其临床特点、组织学特征、鉴别诊断及治疗方法。结果 10例患者,平均年龄43.9岁,8例无明显阳性体征,2例有腰痛症状。CT平扫呈略高密度实性肿块,边界清楚;实质期强化密度最高,排泄期密度下降较少。分别行肾肿瘤剜除术和肾根治性切除术,组织学主要以上皮样分化为主。免疫组化显示肿瘤细胞表达HMB45(+),A103(+),SMA(+),Ki67(+),Desmin(+)。术后10例患者中位随访时间13个月,9例存活,1例术后6个月复发,12个月死亡。结论 EAML临床较少见,术前多无明显症状,确诊需组织病理结合免疫组化,治疗方法可予肿瘤剜除或肾脏切除,预后大多良好,但少数具有潜在恶性,存在恶变可能,术后需要长期密切随访。Objective To investigate the symptoms,diagnosis,imaging and pathological features,treatment,and prognosis of renal epithelioid angiomyolipoma（EAML）.Method Clinical data of 10 cases of renal EAML were retrospectively analyzed,and relevant literature was reviewed.Results Of the 10 cases,7 were male and3 were female,aged between27 to69years（mean 43.9 years）.Eight cases had no positive signs,and2 had waist ache.Unenhanced CT showed slightly hyperdense lump with clear boundary,but no fat could be detected.The mass was enhanced obviously in the parenchymal phase and slightly decreased in excretory phase.Partial nephrectomy of the kidney and radical nephrectomy were performed.The epithelioid cells were large with round or polygon shape,arranged in nests or in sheets.Immunohistochemistolly,tumor cells were positive for HMB45,A103,SMA,Ki67 and Desmin.During the median follow-up of 13 months,9 patients were alive,and the other one had recurrence 6 months and died12 months after operation.Conclusions EAML is a rare disease with no obvious symptoms.It may be easily confused with AML.CT is important in the diagnosis,but a confirmed diagnosis requires pathological and immunohistochemical tests.Surgery is the major treatment method.No standard prognosis exists to date.

关 键 词：肾脏 上皮样血管平滑肌脂肪瘤 病理 免疫组化 预后 

分 类 号：R737.11[医药卫生—肿瘤]