摘要
目的总结结节性硬化症合并肾错构瘤(TSC-RAML)的诊断及治疗进展,提高对该病的认识和诊疗水平。方法回顾性分析总结2014年1月至2017年6月兰州大学第一医院泌尿外科收治的8例TSC-RAML患者的临床资料和诊疗经验,同时结合文献对TSC-RAML的发病机理、诊断及治疗进展进行复习。结果 8例患者除1例妊娠女性患者和1例双RAML破裂并失血性贫血予观察等待处理外,余有5例给予手术治疗,另有1例孤肾患者给予依维莫司药物治疗。随访2～44个月,5例手术治疗患者术后肿瘤有不同程度的复发及残存肿瘤增大,余1例死亡、1例未见肿瘤增大、1例观察期见肿瘤增长。结论需加强多学科会诊制度以避免误诊及漏诊TSCRAML;诊断明确后,需根据患者病情特点拟定个体化医治方案。总体原则是最大限度地保留肾单位,缓解临床症状,预防和治疗RAML破裂出血,延长生存时间。
Objective To summarize the diagnosis and the treatment of tuberous sclerosis complex associated with renal angiomyolipoma （TSC-AML） and to raise awareness and treatment level of the disease.Methods We collected and retrospectively analyzed the clinical data of 8 patients with TSC-AML from January 2014 to June 2017 in the Department of Urology of First Hospital Affiliated to Lanzhou University, and discussed the pathogenesis, the diagnosis and the treatment progress of TSC-AML at the same time.Results One pregnant female patient and 1 patient with hemorrhagic anemia because of rupture of double renal hamartoma weren′t given surgical treatment, but other 5 cases received surgical treatment. The rest 1 case of solitary kidney patients was given everolimus drug treatment. After 2-44 months of follow-up period, 5 patients experienced different degrees of recurrence and residual tumor enlargement after operation, 1 case died, 1 case showed tumor growth during the observation period, and 1 case didn′t.Conclusions The multi-disciplinary consultation system is necessary to avoid misdiagnosis and missed diagnosis. After the diagnosis is confirmed, individualized treatment should be formulated according to patient′s condition. The overall principle is to maximize nephron sparing, to alleviate clinical symptoms, to prevent and treat hamartoma rupture, bleeding, and to prolong survival time.
作者
李开令
李冬梅
白进良
LI Kai-ling;LI Dong-mei;BAI Jin-liang(Department of Urology,the First Hospital,Lanzhou University,Lanzhou 730000,China)
出处
《现代泌尿生殖肿瘤杂志》
2018年第4期206-210,215,共6页
Journal of Contemporary Urologic and Reproductive Oncology
关键词
结节性硬化症
肾错构瘤
Tuberous sclerosis complex
Renal angiomyolipoma
