摘要
目的:探讨肾血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的诊治特点。方法:回顾性分析2013年8月~2017年7月我院收治的16例肾PEComa患者临床资料。男7例,女9例,男女比例1:1.29;年龄21~60岁,平均(41.93±10.96)岁。16例患者中8例表现为腰痛,其中1例有血尿;8例为体检发现。肿瘤最大径1.5~18.0cm,平均(8.00±5.06)cm。肿瘤位于左肾6例,右肾10例。结果:16例患者中9例行保留肾单位手术,7例行根治性肾切除手术。腹腔镜手术9例,机器人手术4例,开放手术3例。免疫组化:抗黑素瘤特异性单抗(HMB45)、抗平滑肌抗体(SMA)、黑色素A抗原(Melan-A)、CD34、波形蛋白(Vimentin)、S-100阳性率分别为93.3%(14/15)、91.7%(11/12)、85.7%(12/14)、85.7%(6/7)、75.0%(6/8)、60.0%(9/15),转录因子E3(TFE-3)和角蛋白(CK)为阴性,病理均诊断为PEComa。16例随访时间3~35个月,未见肿瘤复发及转移。结论:肾PEComa临床罕见,病理为诊断金标准,治疗上首选腹腔镜手术,术后较少发生复发、转移,但对于有恶性倾向或符合恶性标准者需尽早行根治术,并进行严密随访。
Objective： To investigate the diagnosis and treatment of perivascular epithelioid cell tumor of kid- ney （PEComa）. Method： From August 2013 to July 2017, 16 PEComa patients were hospitalized and their data were analyzed retrospectively. There were seven males and nine females with a mean age of （41.93±610.96） years old （range, 21-60）. Eight cases were presented with backache and one of these cases was combined with gross he- maturia, and other eight cases were discovered by health examination. Diameter of tumor was 1.5-18.0 cm, with the average of （8.00-65.06） cm. Six of all cases were observed with tumor in the left kidney, and the other ten were in the right kidney. Result： In 16 cases involved, nine underwent partial nephrectomy and seven patients re- ceived radical nephrectomy. Nine cases underwent laparoscopic surgery, and {our cases underwent surgery with the robot-assisted laparoscope, and the last three cases underwent open surgery. Immunohistochemical staining was positive in HMB45 93.3% （14/15）, SMA 91.7% （11/12）, Melan-A 85.7% （12/14）, CD34 85.7% （6/7）, Vimentin 75.0% （6/8） and S-100 60.0K （9/15）, negative in TFE-3 and CK. Final pathological diagnosis was PEComa. So far, neither recurrence nor metastasis has been detected for 3-35 months. Conclusion： PEComa is a kind of rare tumor. The golden standard is pathological diagnosis. Laparoscopic surgery is the first choice for treatment, after which recurrence and metastasis are rarely encountered. But patients who have a vicious tendency or accord with malignant standard should be treated with radical neDhrectomv and closelv followed up.
作者
李昊
金志波
田向永
丁映辉
黄珍林
汪涛
贾占奎
杨锦建
LI Hao;JIN Zhibo;TIAN Xiangyong;DING Yinghui;HUANG Zhenlin;WANG Tao;JIA Zhankui;YANG Jinjian(Department of Urology, First Affiliated Hospital of Zhengzhou University, Henan Institute of Urology, Zhengzhou, 450052, China~~ Department of Urology, Henan Provincial People＇s Hospital)
出处
《临床泌尿外科杂志》
2018年第3期208-211,共4页
Journal of Clinical Urology
基金
国家自然科学基金项目(编号81570685)
关键词
肾脏
血管周上皮样细胞肿瘤
诊断
治疗
kidney
perivascular epithelioid cell tumor
diagnosis
treatment
