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| 肾上皮样血管平滑肌脂肪瘤临床诊治分析(附7例报告) |
| 盛佳雁1,肖成武1,王林辉2,徐斌3,杨庆1*,高莉4,汪剑5,康钦钦5 |
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(1. 第二军医大学长海医院泌尿外科, 上海 200433;
2. 第二军医大学长征医院泌尿外科, 上海 200003;
3. 上海交通大学医学院附属上海市第九人民医院泌尿外科, 上海 200023;
4. 第二军医大学长海医院病理科, 上海 200433;
5. 第二军医大学长海医院放射科, 上海 200433
*通信作者) |
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| 摘要: |
| 目的 探讨肾上皮样血管平滑肌脂肪瘤（EAML）的临床特点和诊治方法。方法 回顾性分析2012年12月-2014年3月我院收治的7例EAML的临床资料，对其临床、病理、治疗和预后进行探讨和分析。其中男2例，女5例，年龄26-69岁，平均43岁，无合并结节性硬化症，6例为体检发现，1例因肉眼血尿就诊。结果 所有患者均行手术治疗，6例术前被误诊为肾癌，1例合并后腹膜淋巴结以及肺转移，其中有3例行根治性切除术，合并转移者同时行淋巴结清扫术，3例行肾部分切除术；1例术前诊断为间叶性来源肿瘤，但由于肿瘤较大，最终行根治性切除术。术后免疫组化染色检查示肿瘤细胞HMB45和Melan-A阳性，而上皮细胞标记不同分子质量的CK和EMA均为阴性，支持EAML诊断。7例均获得随访，随访时间9-24个月，平均15.1个月。合并转移者1例术后随访提示双肺及肝脏多发转移，其余6例均无瘤存活至今。结论 EAML是一种少见的具有恶性潜能的肿瘤，部分术前难与恶性肿瘤相鉴别，手术方式选择需慎重，术后需要密切随诊。 |
| 关键词: 肾肿瘤 上皮样血管平滑肌脂肪瘤 临床特点 诊断 预后 |
| DOI：10.16781/j.0258-879x.2016.11.1446 |
| 投稿时间：2016-03-08修订日期：2016-05-31 |
| 基金项目:国家自然科学基金（81472771）. |
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| Diagnosis and treatment of renal epithelioid angiomyolipoma: a report of 7 cases |
| SHENG Jia-yan1,XIAO Cheng-wu1,WANG Lin-hui2,XU Bin3,YANG Qing1*,GAO Li4,WANG Jian5,KANG Qin-qin5 |
(1. Department of Urology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China;
2. Department of Urology, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China;
3. Department of Urology, The 9thPeople's Hospital of Shanghai, Shanghai Jiaotong University School of Medicine, Shanghai 200023, China;
4. Department of Pathology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China;
5. Department of Radiology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China
*Corresponding author.) |
| Abstract: |
| Objective To explore the clinical features, diagnosis and treatment of renal epithelioid angiomyolipoma(EAML). Methods 7 cases of renal epithelioed angiomyolipoma(EAML)diagnosed in our unit from December 2012 to March 2014 were reviewed on their clinical and histopathological feathers retrospectively. There were 2 males and 5 females with a mean age of 43(26-69) years. 6 cases were detected in original examination without symptoms and one was admitted for hematuria. None of them had the history of nodular sclerosis. Result All the cases were treated by surgery. 6 cases were diagnosed as renal carcinoma preoperatively and one of them was accompanied by several enlarged lymph nodes in the retroperitoneal region and multiple pulmonarySmetastasis. 4 of the cases were underwent renal radical nephrectomy and 3 were underwent nephron sparing surgery. Immunohistochemcial staining of these tumors showed positive HMB45 and Melan-A but negative CK and EMA which supported EAML. There was no developments in the follow-up of 15.1 months (ranging 9-24 months) in all cases. Conclusions Renal EMAL was a rarely malignant potentiality neoplasm and it is necessary to identify preoperatively. Close follow-up is needed. |
| Key words: kidney neoplasms renal epithelioid angiomyolipoma clinical characteristics diagnosis prognosis |
