肾脏血管周上皮样细胞瘤19例临床诊治分析  

Analysis of clinical diagnosis and treatment of 19 cases of perivascular epithelioid cell neoplasm of the kidney

作　　者：赵奕帆 钱施安 李先创 金恒熙 李煜 赵晓俊[1] ZHAO Yifan;QIAN Shian;LI Xianchuang;JIN Hengxi;LI Yu;ZHAO Xiaojun(Department of Urology,The First Affiliated Hospital of Soochow University,SuZhou 215000,China)

机构地区：[1]苏州大学附属第一医院泌尿外科,苏州215006

出　　处：《现代泌尿生殖肿瘤杂志》2024年第5期260-265,共6页Journal of Contemporary Urologic and Reproductive Oncology

摘　　要：目的探讨肾脏血管周上皮样细胞肿瘤(PEComa)的临床特点。方法回顾性分析2019年1月至2024年4月苏州大学附属第一医院泌尿外科收治的19例肾脏PEComa患者的临床资料,对患者的临床特点、影像学表现、病理特点、治疗及预后进行分析。结果19例患者均行外科手术治疗,15例行肾部分切除术,4例行根治性肾切除术;其中开放手术1例,机器人辅助腹腔镜手术2例,普通腹腔镜手术16例。所有患者病理诊断均为PEComa,免疫组化:黑色素细胞源性标志物HMB-45阳性率84.2%(16/19),黑色素A抗原Melan-A阳性率94.7%(18/19),平滑肌肌动蛋白SMA阳性率100%(18/18),结蛋白Desmin阳性率83.3%(15/18),S100蛋白阳性率53.8%(7/13),CD34阳性率61.5%(8/13),Ki-67指数呈不同程度表达(1%~30%)。19例患者随访时间1~60个月,2例患者因其他原因死亡,其余患者均未见转移或复发。结论肾脏PEComa临床少见,症状及影像表现不典型,病理为诊断金标准,治疗上首选手术切除,大多数患者术后预后良好。Objective To investigate the clinical features of renal perivascular epithelioid cell tumor(PEComa).Methods The clinical data of 19 patients with renal PEComa admitted to the Department of Urology,the First Affiliated Hospital of Soochow University from January 2019 to April 2024 were retrospectively analyzed.The clinical characteristics,imaging findings,pathological features,treatment and prognosis of the patients were analyzed.Results All the 19 patients underwent surgical treatment,including 15 partial nephrectomies and 4 radical nephrectomies.Open surgery was performed on 1 case,Robot-assisted laparoscopic surgery was performed on 2 cases and conventional laparoscopic surgery was performed on 16 cases.Pathological diagnosis of the patients were all PEComas.Immunohistochemical staining was positive in HMB-45 84.2%(16/19),MelanA 94.7%(18/19),SMA 100%(18/18),Desmin 83.3%(15/18),S100 53.8%(7/13),CD34 61.5%(8/13).Ki-67 index expressed in different degrees(1%-30%).The follow-up time of 19 patients was 1-60 months.Two patients died due to other causes,and the other patients did not have metastasis or recurrence.Conclusions Renal PEComa is rare in clinic,with atypical symptoms and imaging manifestations.Pathology is the gold standard for diagnosis.Surgical resection is the first choice for treatment,and most patients have a good prognosis after surgery.

关 键 词：肾脏 血管周上皮样细胞肿瘤 肾肿瘤诊断 免疫组化 肾肿瘤治疗 

分 类 号：R737.11[医药卫生—肿瘤]