双肾血管平滑肌脂肪瘤伴结节性硬化症10例临床分析  

Clinical analyses of bilateral renal angiomyolipomas associated with tuberous sclerosis complex

作　　者：黄钟明[1] 李汉忠[1] 纪志刚[1] 肖河[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院泌尿外科,100730

出　　处：《中华医学杂志》2014年第12期935-937,共3页National Medical Journal of China

摘　　要：目的 探讨双肾血管平滑肌脂肪瘤（RAML）伴结节性硬化症（TSC）的临床诊治特点.方法 分析10例RAML伴TSC患者的临床诊治资料,观察临床症状与病变的大小、部位,比较不同方法治疗后肿瘤的进展情况.患者男4例,女6例,年龄9～44岁,平均23.5岁.5例患者因腰痛、血尿等症状就诊;5例因肾外表现诊断为TSC,行影像学检查发现RAML.结果 10例患者为双侧多发RAML,并符合TSC临床诊断标准,RAML最大直径2～15 cm,平均（7.3±3.5） cm.6例患者存在单侧或双侧RAML破裂出血,肿瘤最大直径平均9.2 cm,其中4例行选择性动脉栓塞（SAE）,2例行止血、抗炎、制动治疗;剩余4例肿瘤无局部症状者密切随访观察.随访9～116个月,4例行SAE治疗者中,1例1年后因再次出血复行SAE,剩余3例无症状复发,肿瘤最大直径平均每年增加1.5 cm,血肌酐平均每年增加3.1μmol/L.行保守治疗或观察的6例患者中,1例因并发肺淋巴管平滑肌瘤,3个月后死于呼吸功能衰竭,剩余5例肿瘤最大直径平均每年增加0.7 cm,血肌酐平均每年增加2.2 μmol/L.2例分别在随访18个月及40个月时出现腰痛等症状,1例经CT证实肿瘤出血行SAE,1例改予雷帕霉素治疗1年后肿瘤明显缩小.结论 合并有TSC的RAML多为双侧多发,在基本除外恶性可能后,SAE因其微创、效果良好且可重复进行,已逐渐成为治疗此类疾病的重要手段;在合并TSC的RAML的药物治疗方面,雷帕霉素显示出一定的疗效.Objective To explore the clinical characteristics,diagnosis,treatment and outcomes of renal angiomyolipoma（RAML） associated with tuberous sclerosis complex （TSC）.Methods The clinical data were analyzed for 10 cases of renal angiomyolipomas associated with tuberous sclerosis.There were 4 males and 6 females with a mean age of 23.5 （9-44） years.Five patients were admitted for flank pain or hematuria.RAML was found by TSC associated imaging examinations for those with extra renal organ involvement.Results Bilateral multiple renal angiomyolipomas were all confirmed on imaging tests and consistent with clinical TSC.The maximal diameter of RAMLs was from 2 to 15 （7.3 ± 3.5） cm.Six patients of tumor hemorrhage had an average maximal diameter of 9.2 cm.Selective arterial embolization （SAE） （n =4） and homeostasis,antiinflamation and immobilization therapy （n =2） were performed.Four asymptomatic cases accepted close observation.During a follow-up period of 9-116 months,one of 4 SAE cases underwent secondary SAE due to rebleeding 12 months later while there was no recurrence of flank pain in other 3 cases.The mean enlargement of maximal diameter was 1.5 cm/year and serum creatinine elevated by 3.1 μmol/L/year.Six cases on conservative medical therapy or under observation had a mean enlargement of maximal diameter of 0.7 cm/year and serum creatinine elevated by 2.2 μmol/L/year.Three cases remained asymptomatic,one died of respiratory failure from TSC-associated pulmonary lymphangioleiomyomatosis,two had flank pain at 16 and 40 months respectively,one underwent secondary SAE and another received rapamysin with marked tumor regression.Conclusions Bilateral multiple RAMLs occur in most TSC patients.After excluding malignancy,SAE becomes an important choice for its mini-invasiveness,efficacy and chances of re-embolization.And rapamysin provides ameliorative treatment for TSC patients.

关 键 词：结节性硬化症 脂肪瘤 栓塞治疗 

分 类 号：R737.11[医药卫生—肿瘤]