小儿肾上腺皮质癌罕见而恶性度高,1983～1993年10月年间本院收治4例,现报告如下。 临床资料 本文4例,男1例,女3例,平均年龄5.12岁(6个月～11岁)。左侧1例,右侧3例,均有功能表达,病程最短3个月,最长3年。3例呈柯兴氏症体征,外阴部色素沉着,有阴毛,血皮质醇均见升高,范围为26～44.8μg/dl。2例行地塞米松抑制试验,大剂量(1.5mg,3次/日)未被抑制。高血压1例,23/15kPa。1例女孩单纯表现为女性男性化,体格粗壮,嗓音低沉,毛孔增粗,有痤疮,有腋毛,外阴部色素沉着,阴毛生长,阴蒂肥大。染色体46XX,血皮 Pediatric adrenocortical cancer is rare and malignant, 1983 to 1993 October in our hospital admitted to 4 cases, are as follows. Clinical data of this article in 4 cases, 1 males and 3 females, with an average age of 5.12 years (6 months to 11 years). 1 case on the left and 3 cases on the right, both of which were functionally expressed with the shortest duration of 3 months and the longest of 3 years. 3 cases of Cushing’s disease signs, genital pigmentation, pubic hair, blood cortisol were seen increased, the range of 26 ~ 44.8μg / dl. 2 routine dexamethasone suppression test, high-dose (1.5mg, 3 times / day) was not inhibited. 1 case of hypertension, 23 / 15kPa. One case of simple performance for women masculine, physical stout, voice low, thick pores, acne, armpit hair, genital pigmentation, pubic hair growth, clitoris hypertrophy. Chromosome 46XX, skin