肾上腺性性征异常的诊断与治疗

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１７α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表现和肿瘤的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１７α羟化酶缺乏的病例,儿童期至青春期不宜应用性激素治疗。

Virilizing and feminizing adrenal syndrome

OBJECTIVE: To inquire into diagnosis, differential diagnosis and treatment of virilizing and feminizing adrenal syndrome especially differential diagnosis between benign and malignant of sex hormone producing adrenal neoplasma and treatment principles of congenital adrenal hyperplasia (CAH). METHOD: Eight cases of CAH and five cases of sex hormone producing adrenal neoplasma were presented during 1986-1996. The former included 3 rare cases of 17 alpha hydroxylase deficiency and others. The latter included 3 cases of feminizing adrenal tumors and 2 cases of virilizing adrenal tumors. RESULT: Weight and diameter of tumor, DHEA, 17-ks and sex hormone levels, appearance of CT imaging, infiltration and metastasis, were closely related to differentiation of benign and malignant tumors. CONCLUSION: Some standards are not absolute because of limited practice, follow-up is very important. Adrenal virilizing and feminizing neoplasms were surgically resected by different incision. Modified subcostal incision is recommended as a best choice for huge adrenal mass. Corticoadrenal hormone treatment of CAH should select different kind of corticoadrenal medicine for different kind of CAH. Treatment of sex hormones is not suitable for children suffered from 17 hydroxylase deficiency until prepuberty.