肾上皮样血管平滑肌脂肪瘤临床分析

摘要

Objective To analyze the clinical and pathological features of epithelioid angiomyolipoma (EAML) in order to improve its diagnosis and treatment.Methods The clinical data of 20 EMAL patients from January 2007 to July 2015 was reviewed retrospectively.The mean age at diagnosis was 44 years old,including 8 males and 12 females.Nineteen tumors were unilateral,with 12 in left kidney and 7 in right kidney,and only 1 case was bilateral.Tumor diameter ranged from 2 cm to 29 cm swith the average diameter was 5.5 cm.Results All of these patients underwent surgery,including 6 patients undergoing radical nephrectomy,6 patients undergoing nephron sparing surgery,2 patients undergoing laparoscopic radical nephrectomy and 6 patients undergoing laparoscopic nephron sparing surgery.Sixteen patients had a mean follow-up of 45 months (range 8-61 months),with 15 patients being alive without disease at the last followup,and 1 patient dying of the tumor.Conclusions EAML accounts for a small proportion of all angiomyolipomas and has aggressive behavior potentially.CT scan may help to differ EAML from renal cell carcinoma,and operation is the common treatment for it.EAML has potential risk ofrecurrence and metastasis and long term follow-up is necessary.%目的 分析肾上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的临床和病理特征,提高对该疾病的诊治水平.方法 回顾性分析2007年1月至2015年7月收治的20例肾EAML患者的临床资料.男8例,女12例.年龄16～63岁,平均44岁.肿瘤位于左侧12例,右侧7例,双侧1例.20例患者均行CT扫描,5例患者行MRI检查.影像学检查示肿瘤直径2～29 cm,平均5.5 cm.结果 20例均行手术治疗,其中行根治性肾切除术6例,保留肾单位手术6例,腹腔镜下根治性肾切除术2例,腹腔镜下保留肾单位手术6例.16例获得随访,随访时间8～61个月,中位随访时间45个月,1例患者因肿瘤导致死亡,其余患者均无肿瘤复发、转移.结论 EAML是较为少见的肾血管平滑肌脂肪瘤,部分缺乏脂肪成分,CT扫描有助于鉴别肾细胞癌,手术切除是主要的治疗方式.EAML绝大多数为良性,少数具有恶性潜能,术后可能出现复发、转移,宜密切随访.