先天性肾上腺皮质增生并发肾上腺皮质肿瘤病例十分罕见。以前报告的病例往往缺乏全面的内分泌学检查或诊断前曾用过皮质类甾醇进行过不充分治疗。作者报告1例,诊断前未经治疗,同时术后作了肿瘤细胞原代单层培养,以了解肿瘤细胞甾类产生受损的特点。 患者为女性,37岁,出生时性别难辨,10岁时出现痤疮、阴毛;12岁身高停止生长。体检呈男性体型,乳房未发育,身高140cm,体重67kg,Bp124 Congenital adrenocortical hyperplasia complicated by adrenal cortical tumors is very rare. Previously reported cases often lack comprehensive endocrinological examinations or inadequate treatment with corticosteroids prior to diagnosis. The authors reported one patient, who had not been treated before diagnosis. At the same time, primary monolayer cultures of tumor cells were performed after surgery to understand the characteristics of tumor cell necrosis. The patient was a woman, 37 years old, and her sex was difficult to distinguish at birth. He had acne and pubic hair at the age of 10 and his height at 12 years stopped growing. Physical examination was male body type, breasts were not developed, height 140cm, weight 67kg, Bp124