结节硬化症合并肾错构瘤的临床诊治分析(附7例报告)  

Clinical diagnosis and treatment of tuberous sclerosis complicated with renal hamartoma(report of 7 cases)

作　　者：师丽娜[1] 谢剑伟 殷波[2] SHI Lina;XIE Jianwei;YIN Bo(Department of Operating Room,Shengjing Hospital Affiliated to China Medical University,Liaoning Shenyang 110004,China;The Second Department of Urology,Shengjing Hospital Affiliated to China Medical University,Liaoning Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院手术室,辽宁沈阳110004 [2]中国医科大学附属盛京医院第二泌尿外科,辽宁沈阳110004

出　　处：《现代肿瘤医学》2021年第7期1179-1182,共4页Journal of Modern Oncology

基　　金：国家自然科学基金(编号:81372725)。

摘　　要：目的:回顾性分析我院诊治的结节硬化症合并肾错构瘤患者的临床资料,并结合分析国内外相关文献资料,以期提高对该病的认识及诊治水平。方法:总结我院诊治的7例结节硬化症合并肾错构瘤患者的临床资料,并检索中国知网、万方、PubMed等数据库,对相关文献进行系统性分析,归纳其临床特点。结果:7例结节硬化症合并肾错构瘤患者,男3例,女4例,年龄20~48岁,平均(34.7±6.23)岁。7例患者均为双侧多发肾错构瘤,肿瘤大小为0.3~31 cm。6例以错构瘤破裂出血伴腰部不适入院,1例以腰部不适入院。2例患者行肾部分性切除术;1例患者行经皮肾动脉栓塞术;2例患者行保守治疗并口服依维莫司治疗;1例患者单纯保守治疗;1例患者口服飞尼妥治疗。至随访截止时,口服飞尼妥的患者肿瘤无明显增大,且并发症相对较少。结论:结节硬化症合并肾错构瘤在临床上属于罕见病例,需要提高对该疾病的临床及影像特征的认识,对患者既往史应该着重注意,加强多学科的合作,遵循结节硬化症最新国际指南进行治疗。对于结节硬化症合并肾错构瘤患者在保留肾功能的情况下,尽量将肿物切尽。依据不同患者的情况选择个体化治疗方案。Objective:To analyze the data of patients with tuberous sclerosis complicated with multiple renal hamartoma diagnosed and treated in our hospital,and to improve the understanding,diagnosis and treatment of the disease.Methods:The data of 7 patients with tuberous sclerosis complicated with multiple renal hamartoma diagnosed and treated in our hospital were summarized,and the databases of CNKI,Wanfang and PubMed were searched,and the related literatures were systematically analyzed and their clinical features were summarized.Results:There were 7 patients with tuberous sclerosis complicated with renal hamartoma,including 3 males and 4 females,aged 20~48 years(mean 34.7±6.23 years old).All 7 patients had bilateral multiple adrenal hamartoma and swelling.The tumor size was 0.3~31 cm.There were 6 patients with ruptured and bleeding hamartoma with lumbar discomfort and 1 patient with lumbar discomfort.2 patients underwent partial renal resection.1 patient underwent transcatheter renal artery embolism.2 patients underwent conservative treatment and oral everolimus.1 patient underwent conservative treatment.1 patient was treated with oral fenito.By the end of follow-up,the tumors of patients with oral fenito were not significantly enlarged,and the complications were relatively few.Conclusion:Tuberous sclerosis complicated with renal hamartoma is a rare case in clinic.It is necessary to improve the understanding of the clinical and imaging features of the disease.Attention should be paid to the past history of the patients.Multi-disciplinary cooperation should be strengthened,and the latest international guidelines for tuberous sclerosis should be followed.For patients with tuberous sclerosis complicated with renal hamartoma,try to cut out the masses while retaining renal function.Individual treatment was selected according to the situation of different patients.

关 键 词：结节硬化症 肾错构瘤 治疗 依维莫司 

分 类 号：R737.11[医药卫生—肿瘤]