单中心985例行肾上腺手术治疗的库欣综合征患者临床特点分析  

Analysis of clinical characteristics of 985 patients with Cushing's syndrome treated with adrenal surgery in a single center

作　　者：赵欣[1] 周家权 刘一鸿[1,3] 樊华 纪志刚[1] 张玉石[1] Zhao Xin;Zhou Jiaquan;Liu Yihong;Fan Hua;Ji Zhigang;Zhang Yushi(Department of Urology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Urology,Hainan General Hospital(Hainan Affiliated Hospital of Hainan Medical University),Haikou 570311,China;People’s Hospital of Zhengzhou,Zhengzhou 450048,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院泌尿外科,北京100730 [2]海南省人民医院(海南医学院附属海南医院)泌尿外科,海口570311 [3]郑州市第七人民医院泌尿外科,郑州450048

出　　处：《中华泌尿外科杂志》2022年第11期818-824,共7页Chinese Journal of Urology

基　　金：中央高水平医院临床科研业务费(2022-PUMCH-B-010)。

摘　　要：目的探讨行肾上腺手术治疗的库欣综合征(CS)患者的临床特点及治疗方式。方法回顾性分析2002年8月至2022年8月于北京协和医院行手术治疗的985例CS患者的临床资料。男210例,女775例;年龄(43.33±13.49)岁,男性年龄高于女性[(45.53±14.39)岁与(42.68±13.16)岁,P=0.016]。本组患者术前选择手术方法的原则为:对于促肾上腺皮质激素(ACTH)非依赖性CS,单侧单发病变行肾上腺肿瘤切除术;单侧多发病变行单侧肾上腺切除术;双侧病变先切除体积较大一侧肿瘤,根据随访结果决定是否行对侧手术。术前怀疑皮质癌患者均行R0切除(肿瘤完整切除,镜下切缘阴性),如肿瘤直径≥6cm,则行开放手术。总结CS患者的临床特点,分析临床症状谱、病因谱,以及相应的治疗方法。结果985例中ACTH非依赖性CS占92.8%(914/985),ACTH依赖性CS占7.2%(71/985)。结合术后病理结果确诊,肾上腺腺瘤最常见,占75.94%(748/985),其次为PBMAH(11.37%,112/985)、库欣病(4.26%,42/985)、PPNAD(3.25%,32/985)、EAS(2.94%,29/985)、皮质癌(2.23%,22/985)。临床表现方面,满月脸、多血质面容、水牛背、向心性肥胖及体重增加较为常见,上述单一症状发生率均>30%。激素分泌方面,ACTH依赖性CS患者皮质醇水平明显高于ACTH非依赖性CS[(38.42±18.21)μg/dl与(22.02±9.48)μg/dl,P<0.001]。亚临床型皮质腺瘤患者(258例)早8时血皮质醇[(16.61±6.78)μg/dl]及24h尿游离皮质醇[103.65(59.83,175.70)μg/24h]低于其他类型患者(P<0.001)。治疗方面,除4例(0.4%)皮质癌因肿瘤较大行开放手术外,余981例均行腹腔镜手术,其中经腹途径占2.24%(22/981),经腹膜后途径占97.76%(959/981)。皮质腺瘤患者中单侧670例(89.57%),双侧78例(10.43%);单侧腺瘤采用腹腔镜肾上腺肿瘤切除/次全切除术;双侧腺瘤者中,4例因CS症状严重同期切除双侧肾上腺肿瘤,余74例均根据肿瘤大小及个数分期行单纯肿瘤切除/肾上腺全切/次全切除术。22�Objective To investigate the etiological spectrum,clinical features,and surgical treatment of patients with Cushing's syndrome(CS)who underwent adrenal surgery.Methods From August 2002 to August 2022,the clinical data of 985 patients with Cushing's syndrome who underwent surgical treatment in the department of urology,Peking Union Medical College Hospital were retrospectively analyzed.There were 210 males and 775 females.The average age was 43.33±13.49 years old.The age of males was older than that of females(45.53±14.39 vs.42.68±13.16 years,P=0.016).The principle of preoperative surgical method selection for patients in this group was described as follow.For adrenocorticotropin(ACTH)independent CS,adrenal tumor resection was considered for unilateral solitary lesions and unilateral adrenalectomy was considered for unilateral multiple lesions.For bilateral lesions,the larger tumor was removed first,and the contralateral operation was decided according to the follow-up results.Patients with suspicion of cortical cancer are subjected to R0 resection,and open surgery was performed if the tumor diameter is≥6 cm.The clinical characteristics of CS patients were summarized.The clinical symptom characters,etiology spectrum and the corresponding selection principles of surgical methods were analyzed.Results Among the 985 cases,ACTH-independent CS accounted for 92.8%(914/985),and ACTH-dependent CS accounted for 7.2%(71/985).According to the postoperative pathological results,ACTH-independent CS was the most common,among which adrenal adenoma was the most common 75.94%(748/985),PBMAH 11.37%(112/985),Cushing's disease 4.26%(42/985),PPNAD 3.25%(32/985),EAS 2.94(29/985),cortical carcinoma 2.23%(22/985).In terms of clinical manifestations,full moon face,bloody face,buffalo back,central obesity and weight gain were more common.The incidence of the above single symptoms was>30%.In terms of hormone secretion,the cortisol level of ACTH-dependent CS patients was significantly higher than that of ACTH-independent CS(P<0.001).Cor

关 键 词：库欣综合征 肾上腺手术 病因谱 临床特点 治疗方式 

分 类 号：R699.3[医药卫生—泌尿科学]