儿童肾上腺皮质癌临床及影像学表现

Clinical features and imaging findings of adrenocortical carcinoma in children

目的观察儿童肾上腺皮质癌(ACC)临床及影像学表现。方法回顾性分析13例经病理确诊的ACC患儿,其中11例术前接受肾上腺CT检查,4例术前接受肾上腺MRI,观察其临床特点及影像学表现。结果ACC患儿中女性多见(10/13,76.92%),临床症状包括明显男性化(9/13,69.23%)、库欣综合征(4/13,30.77%)、阴蒂肥大(3/13,23.08%)、阴毛早现(3/13,23.08%)及腹痛腹部包块(2/13,15.38%)等,多伴血清雄激素升高(10/13,76.92%)。CT显示肾上腺区较大肿物,最大径常>6 cm(7/13,53.85%),呈分叶状(8/13,61.54%),5例(5/11,45.45%)平扫密度不均匀,内见钙化灶;增强后均呈不均匀明显强化(11/11,100%),8例(8/11,72.73%)可见"网络状"改变,并可累及周围组织(2/11,18.18%),局部形成肾静脉或下腔静脉癌栓。MR T2WI中,3例病灶呈稍高信号,1例呈等信号;T1WI 3例呈稍低信号,1例呈等信号;增强后4例病灶均不均匀强化。结论儿童ACC影像学表现具有一定特征性,结合临床有助于诊断。

Objective To observe clinical features and imaging findings of children adrenocortical carcinoma(ACC).Methods Clinical and imaging data of 13 children with ACC confirmed by pathology were retrospectively analyzed.Among them,11 cases received preoperative abdominal CT and 4 cases received preoperative abdominal MR scanning.The clinical and imaging manifestations of ACC were observed.Results Among 13 ACC children,10(10/13,76.92%)were female,and most common clinical manifestations included obvious masculinization(9/13,69.23%),Cushing syndrome(4/13,30.77%),clitoral hypertrophy(3/13,23.08%),pubic hair presbyopia(3/13,23.08%),abdominal pain and abdominal mass(2/13,15.38%).Ten children(10/13,76.92%)had elevated serum androgen.CT showed largeadrenal masses with maximum diameter>6 cm in 7 cases(7/13,53.85%),the lesions were lobulated in 8(8/13,61.54%)cases.Among 13 lesions,5(5/11,45.45%)had uneven density with calcification foci.All lesions unevenly and obviously enhanced after enhancement(11/11,100%),and"network-like"changes were observed in 8 cases(8/11,72.23%).Tumor involvement of surrounding tissue and renal vein or inferior vena cava tumor thrombi were noticed in 2 cases(2/11,18.18%).MR T2 WI showed slightly high signal lesions in 3 cases and medium signal lesion in 1 case,all uneven enhanced after enhancement.Conclusion Imaging manifestations of children ACC had certain characteristics,combining with clinical manifestations were helpful to diagnosis of this disease.