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作者报道1966～1987年间收治的40例巴西儿童肾上腺皮质癌(ACC)。诊断以肿瘤病理检查为标准。38例术后确诊,2例尸解确诊。女性28例,男性12例,平均年龄3.9岁(1天～15.7岁)。生化评估由服用地塞米松前后检查患者24小时尿液的17—羟皮质类固醇(17-OH)、17—酮固醇类(17-KS)和血浆去氢表雄酮硫酸盐(DHA)作出。72.5%的患者于出现临床症状后≥6个月作出诊断,仅11例于6个月内作出诊断。由于肿瘤产生的类固醇影响,37例有男性化的表现,22例出现库兴综合征的体征及生化特征,2例单纯为库兴综含征体征,一些患者男性化明显而掩盖了库兴综合征。临床表现为多毛症(62.5%)、痤疮(47.5%)、高血压(47.5%)、多血症(42.5%)、满月脸(35%)、嗓音粗(32.5%)和癫痫发作(17.5%),
The authors reported 40 cases of adrenocortical carcinoma (ACC) in Brazilian children admitted between 1966 and 1987. The diagnosis is based on the pathological examination of the tumor. 38 cases were diagnosed after operation, and 2 cases were confirmed by autopsy. There were 28 females and 12 males with an average age of 3.9 years (1 day to 15.7 years). Biochemical assessments were made by examining 24-hour urine 17-OH, 17-ketosterols (17-KS) and plasma dehydroepiandrosterone sulfate (DHA) before and after dexamethasone administration. . 72.5% of patients were diagnosed ≥6 months after the onset of clinical symptoms, and only 11 patients were diagnosed within 6 months. Due to the influence of steroids produced by tumors, 37 cases had masculine manifestations, 22 cases showed signs and biochemical characteristics of Cushing’s syndrome, 2 cases were signs of Cushing syndrome alone, and some patients had obvious masculinization that covered up the Cushing synthesis. Sign. The clinical manifestations were hirsutism (62.5%), acne (47.5%), hypertension (47.5%), hyperlipidemia (42.5%), full moon face (35%), coarse voice (32.5%), and seizure (17.5% ),