四川医学Issue(3)：338-342,5.DOI:10.16252/j.cnki.issn1004-0501-2015.03.020
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
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
作者信息
- 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
- 折叠
摘要
Abstract
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 mainland China 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
<br> were limited to mainland China. The related literatures of retrieval were summarized. The main points of disease characteristics and the diagnosis and treatment were analyzed. Results Including the authors’ study, there were 206 patients with an age range of 2 months to 77 years-old. The ratio of male and female was 63/70. The renal hamartoma were exanimated in 96 cases with a size range of 0. 4cm to 45cm( <4cm of 31 cases, 4~10cm of 26 cases,>10cm of 39 cases). The methods of treatment were described in 95 cases, and the patients have underwent medical treatment(MT)in 34 cases(25. 68%), nephron sparing surgery(NSS)in 26 cases (24.32%), radical nephrectomy(RN) in 25 cases(28.38%) and selective renal arterial embolization(SRAE) in 10 cases (13. 51%). There seems to be a trend of small tumor underwent MT, large tumor underwent RN or NSS, and solitary kidney under-went NSS. 25 studies reported patients’ renal function, but only 4 in studies of patients underwent operative interventions reported the renal insufficiency of patients. No paper reported the specific outcomes evaluation, and only 21 studies(28. 38%) reported the follow-up irregularly with a follow-up range of 1 day to 5 years. Conclusion We should improve our level of understanding to the characteristics of TSC associated renal hamartoma, strengthen multidisciplinary cooperation, and follow the international guidelines of TSC. As to the symptomatic TSC associated renal angiomyolipoma, preservation of renal tissue remains a priority. Intervention should be considered for larger tumors, particularly if the patient is symptomatic,taking into account the size of angiomyolipoma, renal func-tion, presence of symptoms, and patient factors, highlighting the need for individualized tailoring of treatment recommendations.关键词
肾疾病/错构瘤/结节性硬化症Key words
kidney diseases/hamartoma/tuberous sclerosis分类
医药卫生引用本文复制引用
廖勇,陶客言,黄建林,李俊,何鹏,邹建华,邱明星,熊国兵..结节性硬化症相关肾错构瘤206例临床分析[J].四川医学,2015,(3):338-342,5.
