肾上腺皮质癌的临床和病理特征:单中心40年回顾性分析  

The clinical and pathological characteristic analyses of adrenocortical carcinoma:a single-center 40-year experience

作　　者：王站 刘义 陈嘉洋 邓建华 张玉石 Wang Zhan;Liu Yi;Chen Jiayang;Deng Jianhua;Zhang Yushi(Deparment of UrologyPeking Union Medical College Hospial,Chinese Academy of Medical Sciences,Beijing 100730,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

出　　处：《中华泌尿外科杂志》2024年第4期265-270,共6页Chinese Journal of Urology

摘　　要：目的探讨肾上腺皮质癌(ACC)患者的临床和病理特点,分析临床特征与Ki-67指数的相关性。方法回顾性分析1984年1月至2024年1月北京协和医院收治的ACC患者的病例资料。纳入标准:①患者接受穿刺或手术,确诊为ACC;或存在典型的皮质多条带受累表现,PET/CT检查提示肾上腺区肿物高摄取,已发生远处转移,并经我院多科会诊,考虑诊断为ACC;②患者接受过胸腹盆CT等检查,能够明确肿物大小及是否发生转移者。排除标准:①缺乏ACC病理诊断或未经我院多科会诊,未明确诊断为ACC;②患者初诊时的症状、体征、辅助检查等信息丢失严重者。收集患者的基线资料(年龄、性别、肿瘤侧别等)、临床表现、内分泌检查、影像学检查和病理检查结果,分析肿瘤最大径、欧洲肾上腺肿瘤研究协作网(ENSAT)分期、肿瘤的功能状态、^(18)F-FDG最大摄取值(SUVmax)与Ki-67指数的关系。结果本研究最终纳入164例患者。男65例,女99例,男:女为1:1.52。中位诊断年龄48(40,58)岁。肿瘤位于左侧71例(43.29%),右侧91例(55.49%),双侧2例(1.22%)。中位肿瘤最大径9(7,12)cm,其中>5cm147例(147/158,93.04%)。162例有ENSAT分期,1、Ⅱ、Ⅱ、Ⅳ期分别为9例(5.56%)、72例(44.44%)、51例(31.48%)、30例(18.52%)。症状可评估的154例中,101例无典型临床表现;53例(34.42%)有典型临床表现,仅有库欣综合征17例(11.04%),库欣综合征合并性征异常5例(3.25%),库欣综合征合并性征异常及原发性醛固酮19例(12.34%),仅有性征异常12例(7.79%)。153例有病理亚型,其中传统型138例(90.20%),嗜酸细胞型13例(8.50%),黏液型和肉瘤样各1例(0.65%)。血管内检出瘤栓24例(14.63%),以下腔静脉瘤栓和肾静脉瘤栓最常见[17例(10.37%)]。中位Ki-67指数为20%(10%,40%),其中>5%占93.13%(122/131)。ENSAT分期Ⅰ、Ⅱ、Ⅲ、Ⅳ期的Ki-67指数分别为20%(10%,35%)、15%(10%,30%)、30%(20%,60%)、30%(20%,60%),Ⅲ期(P=0.0007)、Ⅳ期(P=0.0011)�Objective To investigate the clinical and pathological characteristics of adrenocortical carcinoma(ACC)patients,and to analyze the correlation between clinical features and Ki-67 index.Methods Retrospectively analyze the clinical data of ACC patients admitted into Peking Union Medical College Hospital from January 1984 to January 2024.Inclusion criteria:①The patient underwent puncture or surgery,and was diagnosed as ACC;or those who had typical manifestations of adrenocortical multi-band disorders,extremely high FDG uptake in the adrenal area and distant metastasis,and the diagnosis of ACC was considered after multidisciplinary consultation in our hospital;②The patient had undergone thoracic,abdominal and pelvic CT scan,and could determine the size of the tumor and whether metastasis occurred.Exclusion criteria:①without pathological diagnosis of ACC or no diagnosis of ACC without multidisciplinary consultation in our hospital;②no records of symptoms,signs,examinations and other information at the time of initial diagnosis.The baseline data(age,gender,tumor side,etc.),clinical manifestations,endocrine examination,imaging and pathological examination results of the patients were collected,and the relationship between the maximum tumor diameter,the stage of the European Network for the Study of Adrenal Tumors(ENSAT),the functional status of the tumor,the maximum uptake value of ^(18)F-FDG(SUVmax)and the Ki-67 index was analyzed.Results A total of 164 patients were included in this study.There were 65 males and 99 females,with a male to female ratio of 1:1.52.The median age at diagnosis was 48(40,58)years old.71 tumors were located(43.29%)on the left side with 91 cases(55.49%)on the right side,and 2 cases(1.22%)on both sides.The median tumor diameter was 9(7,12)cm,of which 147 cases(147/158,93.04%)were>5 cm.A total of 162 patients had ENSAT stages,including 9 stages Ⅰ(5.56%),72 stage Ⅱ(44.44%),51 stage Ⅲ(31.48%)and 30 stage Ⅳ(18.52%),respectively.Among the 154 cases with evaluable symptoms,101 had no

关 键 词：肾上腺皮质癌 临床表现 病理特征 库欣综合征 肾上腺源性性征异常 

分 类 号：R736.6[医药卫生—肿瘤]