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目的:探讨肾上腺性性征异常的诊断、鉴别诊断和治疗,特别是分泌性激素的肾上腺肿瘤的良恶性鉴别诊断和各种先天性肾上腺增生疾病的治疗原则。方法:报告1986年～1996年8例先天性肾上腺皮质增生和5例分泌性激素的肾上腺肿瘤的诊治经验。结果:先天性肾上腺皮质增生中有3例17-α羟化酶缺乏症,肿瘤患者包括2例女性男性化、3例男性女性化肾上腺肿瘤。分泌性激素肾上腺肿瘤均经手术切除。结论:分泌性激素的肾上腺皮质肿瘤的重量、直径(DHEA)、尿17酮和血性激素水平、CT表现和肿瘤浸润及转移可作为判断肿瘤良恶性的参考指标。对大体积肾上腺肿瘤首选改良肋缘下切口。先天性肾上腺皮质增生应根据疾病类型而选用不同皮质激素进行治疗,对于17- α羟化酶缺乏的病例。儿童期至青春期不宜应用性激素治疗。
Objective: To investigate the diagnosis, differential diagnosis and treatment of adrenal abnormalities, especially the differential diagnosis of benign and malignant adrenal tumors secreting hormones and the treatment principles of various congenital adrenal hyperplasia diseases. METHODS: The report of 8 cases of adrenal hyperplasia and 5 cases of secretory hormone adrenal tumors from 1986 to 1996 were reviewed. Results: There were 3 cases of 17-α-hydroxylase deficiency in congenital adrenal hyperplasia. The tumor patients included 2 masculine females and 3 females with adrenal tumors. Secretion hormone adrenal tumors were surgically removed. Conclusions: The weight, diameter (DHEA), urinary 17 ketone and serum levels of hormones, CT findings and tumor infiltration and metastasis of adrenocortical tumors secreting hormones can be used as a reference index to judge the benign and malignant tumors. For large volume adrenal tumors preferred modified marginal incision. Congenital adrenal hyperplasia should be based on the type of disease and the choice of different corticosteroids for the treatment of 17-α-hydroxylase deficiency cases. Childhood to adolescence should not be applied hormone therapy.