结节性硬化症相关肾错构瘤206例临床分析  

Clinical Analysis of TSC-associated Renal Angiomyolipomas:A Report of 206 Cases

作　　者：廖勇[1] 陶客言[1] 黄建林[1] 李俊[1] 何鹏[1] 邹建华[1] 邱明星[1] 熊国兵[1] 

机构地区：[1]四川省医学科学院.四川省人民医院泌尿外科放射科,四川成都610072

出　　处：《四川医学》2015年第3期338-342,共5页Sichuan Medical Journal

摘　　要：目的总结诊治伴发结节性硬化症(TSC)的肾错构瘤(AML)患者病例资料,并系统回顾30年国内相关文献资料,以期为认识该病特征、提高对其诊疗水平提供参考。方法总结我院诊治首例伴发结节性硬化症肾错构瘤患者病例资料,同时检索中国生物医学文献数据库(Sinomed)与PubMed,按照预先确定标准对检索文献进行汇总,分析纳入合格文献的研究特征、研究内容,总结该病特点及诊疗要点。结果纳入共计206例患者,年龄2个月~77岁,男女比例63:70。96例患者包含肾脏肿瘤情况,肿瘤大小为0.4cm^45cm,其中<4cm者31例,4~10cm者26例,>10cm者39例。95例包含治疗方法,34例(25.68%)采用保守治疗,26例(24.32%)采用保留肾单位手术,25例(28.38%)采用肾切除术,10例(13.51%)采用选择性肾动脉栓塞。结果倾向于小体积肿瘤保守治疗、大体积行RN或NSS及独肾行NSS。25个研究报告了患者肾功能,而手术中仅4个研究报告患者肾功能不全。没有研究陈述具体结局评估,仅21个研究(28.38%)不规范地报道随访结局,随访时间1天~5年。结论提高对结节性硬化症相关肾错构瘤疾病特征的认识,加强多学科合作,推荐遵循国际化诊疗指南进行规范诊治。对于症状性TSC相关肾AML,保留肾组织的外科干预应当首先考虑。应综合考虑肿瘤大小、肾脏功能、症状表现以及患者情况,实施个体化治疗。Objective The data of first hospitalized patient with Tuberous Sclerosis complex（ TSC）-associated renal an-giomyolipomas managed by our team was analyzed, and the related studies of TSC-associated renal angiomyolipomas of the past 30 years in China's Mainland were systematic reviewed retrospectively, so as to improve the reorganization of corresponding diseases characteristics and the diagnosis and treatment level. Methods Combining analysis of the first case of TSC-associated renal an-giomyolipomas in our hospital, data sources of relevant studies were identified by a search of the Chinese biomedical literature da-tabase（Sinomed）and PubMed to October 20, 2014 with Subject headings combined with free word retrieval. The related researchs

关 键 词：肾疾病 错构瘤 结节性硬化症 

分 类 号：R737.11[医药卫生—肿瘤]