巨大肾血管平滑肌脂肪瘤临床分析(附27例报告)  

Clinical analysis of huge renal angiomyolipoma(report of 27 cases)

作　　者：徐宏博[1] 陈城[1] 张俊[1] 黄玉华[1] 王亮良[1] 胡林昆[1] 袁和兴[1] 侯建全[1] 

机构地区：[1]苏州大学附属第一医院泌尿外科,215006

出　　处：《现代泌尿生殖肿瘤杂志》2017年第3期144-147,共4页Journal of Contemporary Urologic and Reproductive Oncology

摘　　要：目的探讨巨大肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)的临床特点,总结诊治经验。方法回顾性分析2008年1月至2017年1月我院收治的27例巨大RAML患者(肿瘤直径8cm及以上)的临床资料。其中男4例,女23例,年龄16~66岁,平均38岁。肿瘤位于左侧14例,右侧13例。16例有临床症状,其中7例为RAML破裂出血,11例为无症状体检发现。27例术前均行CT检查,3例行MRI检查。结果本组27例均行手术治疗,其中11例行肾切除术,9例行保留肾单位手术,4例行腹腔镜保留肾单位手术,3例行肾癌根治术。7例RAML破裂出血患者中,1例行急诊肾切除术,1例行急诊保留肾单位手术,其余5例待症状稳定,完善相关检查后予手术治疗。根据术后肿瘤大体标本,肿瘤直径为8~27cm,平均13.7cm。术后21例患者获得随访,随访时间1~108个月,中位随访时间42个月,无复发或因肿瘤导致死亡者。结论巨大RAML临床少见,多数具有腰部酸胀不适等临床症状,CT检查为主要的诊断方式,B超可以作为患者随访的首选检查。8cm及以上巨大RAML肿瘤破裂出血风险明显增加,建议积极行手术治疗。巨大RAML以良性为主,预后较好。Objective To discuss the clinical characteristic of huge angiomyolipoma,and to summarize the experience of diagnosis and treatment. Methods The clinical data of 27 patients with huge renal angiomyolipoma（RAML）treated in First Affiliated Hospital of Soochow University from January 2008 to January 2017 were reviewed retrospectively.The mean age of these patients at diagnosis was 38 years old,including 4males and 23 females.There were 14 tumors in the left kidney and 13 in the right.Sixteen patients were symptomatic,including seven patients with rupture hemorrhage.The other 11 patients are asymptomatic.All patients underwent CT scan and three patients received MRI. Results All of these patients underwent surgery,including 11 nephrectomies,nine nephron sparing surgeries,four laparoscopic nephron sparing surgery and three radical nephrectomies.There were seven patients of huge RAML with rupture hemorrhage.Two of them underwent emergency operations including a nephrectomy and a nephron sparing surgeries.The other five patients underwent selective operations.The average diameter of tumors was 13.7cm according to the postoperative pathological specimens.Only 21 patients were followed up for a mean duration of 42 months without tumor recurrence or death. Conclusions Huge RAML（larger than 8cm）is rare and most patients are symptomatic.Ultrasound and CT scan are important in diagnosing with specific manifestation.Surgery is the common treatment for huge RAML for the reason that the risk of rupture hemorrhage is higher.Usually,RAML patients have favorable prognosis.

关 键 词：肾肿瘤 血管平滑肌脂肪瘤 治疗 

分 类 号：R737.11[医药卫生—肿瘤]