不典型肾血管平滑肌脂肪瘤临床诊治分析

Clinical Diagnosis and Treatment of Atypical Renal Angiomyolipoma

目的：提高不典型肾血管平滑肌脂肪瘤（Renal angiomyolipomn，RAML）的诊治水平。方法：回顾分析14例不典型肾缸管平滑肌脂肪瘤的临床和影像学资料，并与病理结果对照。术前5例（35.7％）诊断为RAML，行肿瘤剜除术；2例（14.3％）诊断为肾癌，直接行根治性肾切除术；7例（50％）因术前诊断不明于术中行冰冻切片检查，其中6例诊断为RAML行肾部分切除术，另1例提示为肾癌行肾切除术。结果：本组14例手术均顺利，术后病理诊断均为RAML。术后14例随访6月-8a，肾功能正常。结论：不典型RAML仅靠影像学资料难以准确诊断，应综合分析临床资料、影像学资料，并结合术中冰冻切片检查做出诊断；治疗以保留肾单位的手术为主。

Objective：To summarize the experience of diagnosis and treatment of atypical renal angio- myotipoma （RAML）. Methods：The clinical and imaging data of 12 cases with atypical RAML was analyzed respectively and compared with the pathological results. Five cases （35.7%） diagnosed with RAML before operation accepted tumorectomy; two cases diagnosed with renal carcinoma were performed radical nephrectomy immediately; meanwhile, other seven cases （50%） failed to make a definite diagnosis based on their imaging data received operations, during which the frozen sections of tissues were made and observed under a microscope. Six cases of RAML and one of renal carcinoma were confirmed by intraoperative pathological examination and accepted heminephrectomy and nephrectomy respectively. Results： These patients underwent operations smoothly. During the follow up periods of six months to eight years, all the patients＇ renal function was normal. Conclusions ： It is difficult to make a definite diagnosis of atypical RAML by depending on imaging data. Surgeons will comprehensively analyze clinical data, imaging characteristics and intraoperative pathological results of frozen sections. The nephron-sparing surgery was the best operative management.