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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>300 g,>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>300 g,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。
Objective To investigate the clinicopathological characteristics and differential diagnosis of children with adrenocortical carcinoma (ACC). Methods 21 cases of adrenocortical carcinoma of children were retrospectively reviewed and HE staining and immunohistochemical staining were performed. Results 21 cases of children aged 1 to 12 years old, average 4.9 years old, male to female ratio of 1: 3.2, 85.7% of children with signs of increased cortisol hormone. Tumor volume is generally larger, 52.4% of the cases of adrenal tumor weight> 300 g,> 80% of the visible microscopic clear cell sparse, pathological mitosis, infiltration of the veins and the capsule. Immunohistochemistry: 21 cases of vimentin, Syn and PCNA (+), 7 cases of CK (AE1 / AE3) (+), 11 cases of Ki-67 (+), CEA, EMA (-), PCNA positive rate was 28.8% ~ 98.2%, Ki-67 is 1.1% ~ 23.1%. Conclusion Adrenocortical cancer in children with high malignant, poor prognosis, more common in girls, most patients with signs of increased cortisol hormone. Diagnosis of ACC in children, the majority of the tumor weight> 300 g, tumor tissue sparse cells, pathological mitosis, infiltration of the veins and the capsule is a relatively important indicator. PCNA positive rate> 30%, Ki-67 positive rate> 10% of the diagnosis of ACC in children with ancillary effects.