肾上皮样血管平滑肌脂肪瘤2例报告并文献复习  

Renal epithelioed angiomyolipoma:a report of 2 cases and literature review

作　　者：丰琅[1] 宋健[1] 张道新[1] 

机构地区：[1]首都医科大学附属北京友谊医院泌尿外科,北京100050

出　　处：《现代泌尿外科杂志》2016年第6期448-451,共4页Journal of Modern Urology

摘　　要：目的提高对肾上皮样血管平滑肌脂肪瘤的认识,探讨其诊治方法。方法报告2例经本院收治的肾上皮样血管平滑肌脂肪瘤患者的临床资料,回顾性分析其临床特征及影像学表现。2例患者有1例有腹痛症状,1例无临床症状。术前超声检查均提示混合回声占位,占位可见血流信号。CT检查呈现不均匀强化。术前均诊断为肾脏恶性肿瘤。分析2例患者临床资料并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果 2例患者均接受根治性肾切除手术治疗。术后病理提示肾上皮样血管平滑肌脂肪瘤,肿瘤均侵及脉管且有瘤栓形成。免疫组化染色HMB-45及Melan-A均阳性。2例患者分别获随访3个月及18个月,未见肿瘤复发。结论肾上皮样血管平滑肌脂肪瘤临床少见。其生物学行为可呈现恶性表现。确诊需要依靠病理结合免疫组化。手术是主要的治疗方法,在对肿瘤恶性行为进行判断时应该综合考虑病例临床特征、病理特点以及分子生物学指标。应该对患者进行长期密切随访。Objective To discuss the diagnosis and treatment of renal epithelioed angiomyolipoma（REAML）.Methods Clinical data of 2 cases of REAML were reported,and the clinical characteristics and image findings were retrospectively analyzed.One patient had abdominal pain and the other presented no special clinical manifestations.The two patients had undergone ultrasonography and showed a mixed echo mass,with obvious blood flow signal.The CT scan showed heterogeneous enhancement at the arterial phase scan.Both cases were diagnosed as malignant renal tumor before surgery.The related articles were searched in Pubmed and CBM databases and data collected were reviewed.Results Both patients underwent radical nephrectomy.Pathological tests confirmed REAML.The vessels were invaded and tumor thrombus was observed.The immunohistochemical staining of HMB-45 and Melan-A were positive.During the follow-up of 3to18 months,no tumor recurrence occurred.Conclusions Renal epithelioed angiomyolipoma is a rare disease.The biological behavior shows malignancy.Pathology and immunohistochemistry are important to confirm the diagnosis.Surgery is the major treatment approach.The clinical manifestations,pathological characteristics and molecular biology markers should be synthesized for the evaluation of tumor malignancy.It is necessary to follow up patients for a long term.

关 键 词：肾上皮样血管平滑肌脂肪瘤 电子计算机X线断层摄影术 手术 预后 

分 类 号：R737.11[医药卫生—肿瘤]