肾脏上皮样血管平滑肌脂肪瘤的临床特点分析  

Clinical features of renal epithelioid angiomyofipoma

作　　者：方辉龙 杨元兴 李黎明[1] FANG Hui-long;YANG Yuan-xing;LI Li-ming(Department of Urology,General Hospital of Tianjin Medical University,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院泌尿外科,天津300052

出　　处：《天津医科大学学报》2020年第2期166-170,187,共6页Journal of Tianjin Medical University

摘　　要：目的:探讨肾脏上皮样血管平滑肌脂肪瘤(EAML)的临床特点。方法:回顾性分析在2012年6月-2018年11月收治的10例EAML患者的临床资料,对其临床表现、影像特点、病理特征、治疗方法和预后进行分析。结果:10例患者中,女性9例,男性1例,平均年龄36岁,3例在体检时发现,7例有阳性体征。彩超可见囊实性或不均匀实性病灶;CT平扫呈低密度或稍高密度影,可见不同程度的强化;MRI在T2WI上表现为低信号,呈不均匀强化。8例行肾脏部分切除术,1例行根治性肾脏切除术,1例行肾脏切除术。术后病理8例诊断为EAML;2例病理诊断为肾脏血管平滑肌脂肪瘤(AML),部分呈EAML。免疫组化染色HMB-45(+)、Melan-A(+)、SMA(+)、CK(-)。术后随访时间7~50个月,平均随访时间28个月,暂未发现复发或远处转移。结论:EAML在临床上较少见,早期症状不明显,确定诊断依靠术后病理HE染色和免疫组化染色。EAML具有恶性潜能,治疗主要以手术切除为主,目前预后尚无统一认识,需要长时间密切随访。Objective:To explore the clinical features of renal epithelioid angiomyolipoma(EAML).Methods:The clinical data of 10 patients with EAML admitted from June 2012 to November 2018 were investigated,retrospectively.The clinical manifestations,imaging features,pathological features,treatment methods and prognosis were analyzed.Results:Among the 10 patients,9 cases were female and 1 case was male,with an average age of 36 years.Three patients were found in physical examination,and 7 patients had positive signs.Cystic solid or heterogeneous lesions were scan by color ultrasound;CT plain scan showed low density or slightly high density shadow,and the CT enhancement scan showed different degrees enhancement;low signal and uneven enhancement showed on T2WI by MRI.Eight patients were performed partial nephrectomy,and 1 patient was treated with radical nephrectomy,and 1 patient was treated with nephrectomy.Postoperative pathology indicated that 8 patients were diagnosed as renal epithelioid angiomyolipoma,and 2 patients were diagnosed as renal angiomyolipoma which showed partial epithelioid angiomyolipoma features.Immunohistochemical staining results of the tumor showed that HMB-45(+)、Melan-A(+)、SMA(+)、CK(-).Postoperative follow-up time was 7 to 50 months,with an average follow-up time of 28 months.No recurrence or distant metastasis cases were found.Conclusion:EAML is rare in clinical practice and early symptoms of which are not obvious.The definitive diagnosis of EAML depends on postoperative pathological HE staining and immunohistochemical staining.EAML has malignant potential,and the main treatment of which is surgical resection.There are no unified understanding of the prognosis until now,which requires long-term and close follow-up.

关 键 词：肾脏上皮样血管平滑肌脂肪瘤 病理 治疗 预后 

分 类 号：R692.9[医药卫生—泌尿科学]