肾脏上皮样血管平滑肌脂肪瘤伴静脉瘤栓7例报告及文献复习  

Renal epithelioid angiomyolipoma with venous thrombus:a report of 7 cases and literature review

作　　者：杨济宇 戴津东 刘振华[1] 刘志洪[1] 林天海[1] 张朋[1] 魏强[1] 李响[1] 曾浩[1] 陈铌[2] 聂玲[2] 张孟尼 沈朋飞[1] YANG Jiyu;DAI Jindong;LIU Zhenhua;LIU Zhihong;LIN Tianhai;ZHANG Peng;WEI Qiang;LI Xiang;ZENG Hao;CHEN Ni;NIE Ling;ZHANG Mengni;SHEN Pengfei(Department of Urology,West China Hospital,Sichuan University,Chengdu 610041,China;Department of Pathology,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院泌尿外科,四川成都610041 [2]四川大学华西医院病理科,四川成都610041

出　　处：《现代泌尿外科杂志》2022年第5期394-399,共6页Journal of Modern Urology

基　　金：四川省科技厅重点研发项目基金(No.2021YFS0119)。

摘　　要：目的报告7例肾上皮样血管平滑肌脂肪瘤(EAML)合并静脉瘤栓病例,并探讨针对该病患者的诊疗方法。方法回顾性分析2009年11月-2021年4月就诊于四川大学华西医院的7例肾脏EAML合并静脉瘤栓患者的资料,分析患者的临床表现、临床与病理学特性、诊断与鉴别诊断及外科治疗方式。术后定期对患者进行随访。结果7例患者中,术前影像示2例患者肿瘤内可见脂肪密度影,另外5例则无明显脂肪组织;影像学诊断5例倾向于肾恶性肿瘤,2例倾向于肾错构瘤;7例患者均行根治性肾切除+静脉瘤栓取出术;术后组织病理学均证实为EAML及静脉内瘤栓形成。2例患者失访,除1例患者术后2个月出现肺部瘤栓外,其余4例患者术后未发现复发或转移。结论部分EAML有恶性倾向,表现出瘤体侵犯肾静脉或下腔静脉并形成瘤栓。EAML影像学表现容易与肾细胞癌相混淆,临床医生应加强对此类疾病的辨别。手术切除肿瘤并取出瘤栓是治疗EAML合并瘤栓的唯一有效方法。此类疾病术后存在复发转移的风险,应加强术后随访。Objective To report the clinical characteristics of 7 cases of renal epithelioid angiomyolipoma(EAML)complicated with venous thrombus and to explore the diagnosis and treatment.Methods Clinical data of 7 patients with renal EAML complicated with venous thrombus treated in our hospital during Nov.2009 and Apr.2021 were retrospectively analyzed.The clinical manifestations,pathological features,diagnosis,differential diagnosis,and surgical treatment were analyzed.Patients were followed up regularly after surgery.Results Preoperative imaging showed fat density shadow in 2 patients and no obvious adipose tissue in the other 5 patients.Malignant tumor was suspected in 5 cases and renal hamartoma in 2 cases.All 7 patients underwent radical nephrectomy plus thrombectomy.Postoperative histopathology confirmed EAML and intravenous thrombus formation.Except 2 patients who were lost to follow-up,and 1 who had pulmonary tumor embolism 2 months after surgery,the other 4 cases had no recurrence or metastasis.Conclusion EAML has a malignant tendency,showing tumor invasion of renal vein or inferior vena cava.Imaging findings can easily confuse EAML with renal cell carcinoma,so clinicians should be very careful in the identification of this disease.Surgical resection of tumor and removal of venous thrombus is the only effective treatment.Postoperative follow-up is necessary to detect recurrence and metastasis.

关 键 词：肾脏肿瘤 上皮样血管平滑肌脂肪瘤 病理诊断 静脉瘤栓 影像学 手术 

分 类 号：R737.11[医药卫生—肿瘤]