小儿原发性肾上腺皮质腺癌和原发性肾上腺皮质腺瘤临床特征、影像学表现及其误诊分析  

The Imaging Manifestations,Clinical Characteristics and Misdiagnosis of Primary Adrenocortical Carcinoma and Primary Adrenocortical Adenoma in Children

作　　者：徐守军[1] 杨春兰[2] 曹娟[3] 李鹏[1] 刘龙平 谢丽春 罗娜[1] 裴妮慧 XU Shoujun;YANG Chunlan;CAO Juan(Department of Radiology,Shenzhen Children's Hospital,Shenzhen,Guangdong Privonce 518026,P.R.China)

机构地区：[1]深圳市儿童医院放射科,518026 [2]深圳市儿童医院血液肿瘤科,518026 [3]深圳市儿童医院病理科,518026 [4]广州医科大学附属第三医院儿科,510530

出　　处：《临床放射学杂志》2024年第6期995-1003,共9页Journal of Clinical Radiology

基　　金：广东省高水平临床重点专科(编号:SZGSP012);广东省基础与应用基础研究基金项目(编号:2022A1515111121);深圳市医学重点学科建设经费(编号:SZXK034);深圳市医疗卫生三名工程项目(编号:SZSM202011005);深圳市基础研究专项(自然科学基金)基础研究面上项目(编号:JCYJ20220530155616038)资助。

摘　　要：目的观察小儿原发性肾上腺皮质腺癌(PAC)和原发性肾上腺皮质腺瘤(PAA)的临床特征及影像学表现,并对误诊病例进行分析,以提高诊断正确率。方法回顾性分析3例PAC和5例PAA患儿病例资料。3例PAC均为女性,年龄1岁1个月~12岁11个月,平均(5.06±5.56)岁;5例PAA中男2例,女3例,年龄9个月~14岁,平均(4.92±4.78)岁。3例PAC术前均行CT平扫及增强扫描,其中1例同时行MRI平扫及增强扫描。5例PAA术前3例行CT平扫及增强扫描,另有1例仅有CT平扫,1例无CT检查,但此2例术前均行MRI平扫及增强扫描。结果3例PAC,左侧1例,右侧2例;5例PAA,左侧3例,右侧2例。病灶呈类圆形5例,椭圆形2例,分叶状1例。PAC病灶最大径线为43~61 mm,平均(46.67±10.53)mm;PAA病灶最大径线为21~39 mm,平均(34.60±8.31)mm。3例PAC,首次就诊原因为阴蒂肿大1例,颜面皮疹、多食1例,偶发1例。CT检查均以等密度影为主,内见囊变/坏死区,且体积越大越明显。1例内含钙化,均未见出血及骨质破坏。增强扫描病灶均呈不均匀强化,各期强化程度均低于肾实质,其中1例渐进性强化,另外2例以静脉期强化最为明显,延迟期有所减退;瘤内均有细小强化血管影穿行;囊变及坏死区无强化。左肺下叶转移、下腔静脉内见充盈缺损1例。1例PAC术前行MRI检查,以T_(1)WI等或稍低、T_(2)WI稍高信号为主(与肝脏相比),内散在不规则片状T_(2)WI高信号囊变/坏死,DWI明显扩散受限。反相位信号稍减低,信号强度指数(SII)约为9%。增强扫描不均匀明显强化。病灶占位效应明显,邻近肝实质及右肾上极明显受压。下腔静脉内见类圆形充盈缺损;腹膜后淋巴结增大。5例PAA,首次就诊原因为进行性肥胖1例,乳房增大1例,腹痛、便血1例,偶发2例。4例PAA术前CT检查(其中1例仅行平扫)均以等密度影为主,内见囊变/坏死区,且体积越大越明显;但均未见钙化、出血及骨质破坏。增强扫描病灶均呈不Objective The purpose of this study was to highlight the imaging findings of primary adrenocortical carcinoma(PAC)and primary adrenocortical adenoma(PAA)on CT and MRI,to summarize the clinical characteristics and the factors causing misdiagnosis.Methods We retrospectively analyzed the Imaging characteristics of the patients with histologically confirmed PAC and PAA diagnosed.All the three patients with PAC were female,aged from 1 year and 1 months to 12 years and 11 months,with an average age of(5.06±5.56)years;among the five patients with PAA,there were 2 males and 3 females,aged from 9 months to 14 years,with an average age of(4.92±4.78)years.Preoperative plain and enhanced CT were performed in all 3 cases with PAC,Preoperative plain and enhanced MRI were performed in 1 case.Preoperative plain and enhanced CT were performed in 3 cases with PAA.In addition,only CT plain scan in 1 case,and CT was not performed in the other case,however,preoperative plain and enhanced MRI were performed in these 2 cases.Results 3 cases of PAC,1 was located in the left side and 2 in right side.5 cases of PAA,3 were located in the left side,2 was in right side.The lesions were circular in 5 cases,elliptical in 2 cases and lobulated in 1 case.The maximum diameter of PAC lesions ranged from 43 mm to 61 mm,with an average of(46.67±10.53)mm,The maximum diameter of PAA lesions ranged from 21 mm to 39 mm,with an average of(34.60±8.31)mm.Among the 3 patients with PAC,the reasons for the first visit were clitoris enlargement in 1 case,facial rash in 1 case,overeating in 1 case,and occasional occurrence in 1 case.CT examination mainly showed isodensity,cystic lesions/necrotic areas were seen,and the larger the volume,the more obvious.Calcification was found in 1 case,and no bleeding or bone destruction was observed.All lesions showed uneven enhancement on enhanced scan,and the enhancement degree of each stage was lower than that of renal parenchyma.Progressive enhancement in 1 case,and most obvious in the venous stage,decreased in the de

关 键 词：肾上腺皮质腺癌 肾上腺皮质腺瘤 计算机体层成像 磁共振成像 误诊分析 

分 类 号：R736.6[医药卫生—肿瘤]