肾上皮样血管平滑肌脂肪瘤临床诊治分析(附7例报告)

肾上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)是血管平滑肌脂肪瘤(angiomyolipoma,AML)的亚型,为一种罕见的间叶源性肿瘤.因EAML短期内即会出现其他器官转移,呈现侵袭性生物学行为,逐渐引起人们对它的重新认识,因而被认为是一种具有恶性潜能的肿瘤1-2].本研究回顾性分析了2012年12月至2014年3月第二军医大学长海医院泌尿外科收治的7例EAML患者的临床特点及诊治经验,现报告如下.

Diagnosis and treatment of renal epithelioid angiomyolipoma: a report of 7 cases

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.