10例肾血管周上皮样细胞肿瘤临床病理分析

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摘要目的探讨肾血管周上皮样细胞肿瘤的临床病理特点。方法随访2013年6月～2015年9月我院收治的经病理确诊的10例肾血管周上皮样细胞肿瘤患者，回顾性分析其临床病理特点并总结治疗方案。结果10例肾血管周上皮样细胞肿瘤患者中，男女比为1∶9；平均年龄为（50.9±19.5）岁，中位年龄为40.5岁。10例患者均经手术治疗；平均随访时间（18.6±8.2）个月，中位随访时间19.5个月；无瘤生存7例，2例带瘤生存，1例死亡；肿瘤最大径为（5.3±2.8）cm，中位直径5.1 cm；合并出血或坏死5例。9例为单纯的肾血管周上皮样细胞肿瘤，1例合并肾盂尿路上皮癌。所有病例的HMB45和（或）Melan-A表达均呈阳性，Ki67平均（7.0±6.1）%，中位阳性率为5.0%。结论肾血管周上皮样细胞肿瘤临床不易诊断，主要依靠病理确诊，治疗方式倾向于手术切除为主，预后大多较好。对于不能切除或保守治疗的患者，应密切复查随访。

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	林乐
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关键词 ：血管周上皮样细胞肿瘤, 肾上皮样血管平滑肌脂肪瘤, 肾肿瘤, 肿瘤治疗, 临床病理特点

Abstract：Objective To explore the clinicopathologic characteristics of renal perivascular epithelioid cell tumors (PEComas).Methods 10 cases of renal PEComas confirmed by pathology from June 2013 to September 2015 in our hospital were followed up,the clinical pathological features were analyzed retrospectively and the treatment plan was summarized.Results In the 10 cases of renal PEComas,male to female ratio was 1∶9,their average age was(50.9±19.5) years old,with a median age of 40.5 years old.All 10 cases were treated by surgical resection.The average time of follow-up was(18.6±8.2)months,with a median follow-up time of 19.5 months.Disease-free survival were found in 7 cases,while two cases survived with tumors and one died.Maximum tumor length was(5.3±2.8)cm,with a median length of 5.1 cm,hemorrhage or necrosis were confirmed in five of them.9 cases were diagnosed as pure renal PEComa.One patient was found accompanied with urothelial cell carcinoma of the renal pelvis.All cases presented positively labeled with HMB45 and/or Melan-A,the average positive ratio of Ki67 was(7.0±6.1)%,with its medium 5.0%.Conclusion As clinical diagnose of renal PEComa is quite difficult,its diagnose mainly relies on pathological findings.Surgical resection is tended to treat this kind of patients.Most of them results well outcomes.For the tumors unable to removed or dealt with conservative treatment,this kind of patients should be closely followed-up.

Key words： Perivascular epithelioid cell tumors,Renal epithelioid angiomyolipoma Renal tumor Tumor therapy Clinicopathologic characteristic

基金资助:福建省自然科学基金资助项目（2016J05177）

通讯作者: 魏永宝（1984-），男，博士，研究方向：泌尿生殖系肿瘤，擅长泌尿系肿瘤微创治疗及临床基础研究，主持及参与省部级课题共5项，发表论文20余篇，其中SCI论文10余篇，获得2次国家研究生奖学金，并获得湖南省优秀毕业研究生称号

作者简介: 林乐（1973-），男，硕士，副主任医师，研究方向：泌尿系肿瘤，擅长泌尿系肿瘤微创手术及临床研究，发表中文论文10余篇

引用本文:

林乐；魏永宝；李涛；陈梓甫；高祥勋. 10例肾血管周上皮样细胞肿瘤临床病理分析[J]. 中国当代医药, 2017, 24(1): 4-8转15.
LIN Le；WEI Yong-bao；LI Tao；CHEN Zi-fu；GAO Xiang-xun. Clinicopathologic analysis of 10 cases of renal perivascular epithelioid cell tumors. 中国当代医药, 2017, 24(1): 4-8转15.

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