膀胱副神经节瘤的诊疗选择:单中心回顾研究  

Diagnosis and treatment options for paraganglioma of the urinary bladder:a retrospective single center study

作　　者：赖鹏 林登强 刘宇军 蒋晶晶 张晶 张建平 张立 许明 林宗明 郭剑明 LAI Peng;LIN Dengqiang;LIU Yujun;JIANG Jingjing;ZHANG Jing;ZHANG Jianping;ZHANG Li;XU Ming;LIN Zongming;GUO Jianming(Department of Urology,Zhongshan Hospital,Fudan University,Xiamen,Fujian,361015,China;Department of Urology,Zhongshan Hospital,Fudan University;Department of Endocrinology,Zhongshan Hospital,Fudan University)

机构地区：[1]复旦大学附属中山医院(厦门)泌尿外科,福建厦门361015 [2]复旦大学附属中山医院泌尿外科 [3]复旦大学附属中山医院内分泌科

出　　处：《临床泌尿外科杂志》2023年第7期487-491,共5页Journal of Clinical Urology

基　　金：厦门市医疗卫生科技计划项目(No:3502Z20194029,3502Z20209054)。

摘　　要：目的:探讨提高膀胱副神经节瘤(paraganglioma of the urinary bladder,PUB)术前诊断率、改善围手术期管理及治疗的方案。方法:回顾性分析2009年1月—2021年9月复旦大学附属中山医院泌尿外科收治的17例PUB患者的临床资料,分析术前误诊的原因及治疗选择。结果:17例患者中,男10例,女7例;平均年龄(53.5±18.8)岁。表现为排尿/憋尿时心悸、出汗、头痛头晕症状3例(17.6%),血尿5例(29.4%),无症状9例(52.9%);肿瘤中位直径17.0(15.0,25.0)mm;嗜铬细胞瘤相关生化检测5例(29.4%),阳性率40.0%(2/5);CT等影像学确诊率23.5%(4/17);膀胱镜检查14例,其中3例活检确诊。术前确诊率41.2%(7/17)。术前确诊者与术后确诊者比较,平均年龄较小[(40.0±20.1)岁vs(62.9±10.8)岁,P<0.05]、肿瘤较大[33.0(23.5,43.0)mm vs 15.0(14.3,15.8)mm,P<0.05],有症状者比率(85.7%vs 20.0%,P<0.05)及影像学确诊率(57.1%vs 0,P<0.05)均较高。10例(58.8%)行膀胱部分切除术,7例(41.2%)行经尿道膀胱肿瘤电切术(TURBt)。术后病理均为PUB,其中恶性3例(17.6%);基因检测琥珀酸脱氢酶复合体亚基B(SDHB)突变率21.4%(3/14)。与TURBt组比较,膀胱部分切除术组年龄较小[(43.6±17.5)岁vs(67.6±9.3)岁,P<0.05]、肿瘤较大[25.0(20.5,38.3)mm vs 15.0(13.0,15.5)mm,P<0.05]、术前确诊率较高(70.0%vs 0,P<0.05),术后住院时间和随访时间差异无统计学意义(P>0.05)。平均随访时间(30.2±14.2)个月,2组均未出现复发或转移。结论:对于年轻的、有排尿伴随症状(如心悸、头痛、出汗等)、影像学或膀胱镜提示肿瘤来自膀胱黏膜下或肌层的患者,应警惕PUB的可能性,对其进行恰当的生化检测是提高术前诊断率的重要方法。膀胱部分切除是首选治疗方法,对于较小的、表浅的肿瘤,TURBt也可作为备选方案。不同术式的长期疗效及其与SDHB突变的关系尚需长期的、多中心的研究观察。Objective To explore the solution of improving the preoperative diagnosis rate,perioperative management and treatment of paraganglioma of the urinary bladder(PUB).Methods The clinical data of 17 patients with PUB from January 2009 to September 2021 at Department of Urology,Zhongshan Hospital,Fudan University were reviewed,and the reasons for preoperative misdiagnosis and treatment options were analyzed.Results There were 17 patients,including 10 males and 7 females.The average age was(53.5±18.8)years.The symptoms included postmicturition palpitation,sweating,headache or syncope in 3 cases(17.6%),hematuria in 5 cases(29.4%)and no symptoms in 9 cases(52.9%).The median tumor diameter was 17.0(15.0,25.0)mm.Biochemical tests of catecholamine metabolites were performed in 5 patients(29.4%),with a positive rate of 40.0%(2/5).The diagnosis rate of CT and other imaging was 23.5%(4/17).Cystoscopy was performed in 14 cases,of which 3 were confirmed by biopsy.The total preoperative diagnosis rate was 41.2%(7/17).Compared with those diagnosed after surgery,the median age of preoperative diagnosed patients was younger([40.0±20.1]years vs[62.9±10.8]years,P<0.05)and the tumor was larger(33.0[23.5,43.0]mm vs 15.0[14.3,15.8]mm,P<0.05).The proportion of symptomatic patients(85.7%vs 20.0%,P<0.05),and the imaging diagnosis rate(57.1%vs 0,P<0.05)were higher.Ten cases(58.8%)underwent partial cystectomy and 7 cases(41.2%)underwent TURBt.PUB was confirmed by postoperative pathology,among which 3 cases(17.6%)were malignant.The mutation rate of SDHB was 21.4%(3/14).Compared with TURBt group,the median age of partial cystectomy group was younger([43.6±17.5]years vs[67.6±9.3]years,P<0.05),the tumor was larger(25.0[20.5,38.3]mm vs 15.0[13.0,15.5]mm,P<0.05),and the preoperative diagnosis rate was higher(70.0%vs 0,P<0.05).There was no significant difference in postoperative hospital stay or follow-up time(P>0.05).The average follow-up time was(30.2±14.2)months,and no recurrence or metastasis occurred in either group.Conclusion For young p

关 键 词：副神经节瘤 嗜铬细胞瘤 膀胱 诊断 手术 

分 类 号：R737.1[医药卫生—肿瘤]