儿童肾上腺皮质癌21例临床病理分析

目的 探讨儿童肾上腺皮质癌（ACC）的临床病理学特点及鉴别诊断。方法 对21例儿童肾上腺皮质癌回顾病史，并进行HE染色及免疫组化标记。结果 21例儿童发病年龄1～12岁，平均4．9岁，男女之比为1：3．2，85．7％的患儿合并有皮质醇激素增高的体征。肿瘤体积普遍较大，52．4％的病例肾上腺肿瘤重量〉300g，〉80％的肿瘤镜下可见透明细胞稀少，病理性核分裂，静脉及被膜的浸润。免疫组化：21例vimentin、Syn和PCNA（＋），7例CK（AE1／AE3）（＋），11例Ki-67（＋），CEA、EMA（-），其中PCNA阳性率为28.8％～98．2％，Ki-67为1．1％～23．1％。结论 儿童肾上腺皮质癌恶性度高，预后差，多见于女童，患者大多合并有皮质醇激素增高的体征。在儿童ACC的诊断中，肿瘤的重量多数〉300g，瘤组织透明细胞稀少、病理性核分裂、静脉和被膜的浸润是相对重要的指标。PCNA阳性率〉30％、Ki-67阳性率〉10％对儿童ACC的诊断有辅助作用。

Adrenal cortical carcinoma in children: a clinicopathological analysis of 21 cases

Objective To study the clinicopathologic features of adrenal cortical carcinoma(ACC) in children.Methods 21 cases of children ACC were studied by light microscopy,immunohistochemistry and analysis of clinical features. Results The mean age of the 21 patients at diagnosis was 4.9 years(range 1-12 years).77% patients were female and most of them had the functional tumors(86%).The tumors more commonly had a large size,52% more than 300?g in weight.Histologic findings included lack of clear cell,atypical mitotic figures,invasion of venous structures and capsule in 80% patients.Immunohistochemically,the tumor cells were positive for vimentin,Syn and PCNA(21 cases),CK(AE1/AE3)(7 cases) and negative for CEA and EMA. Conclusions Pediatric patients with ACC are more commonly seen in the female.Most of them have the functional tumor.The size of tumors,lack of clear cell,atypical mitosis,vascular and capsule invasion,PCNA and Ki-67 values are predictors for malignant behavior of the tumor.