Ojima M, Fukuchi S
Gan To Kagaku Ryoho. 1983 Oct;10(10):2093-102.
Adrenocortical tumors can be divided into two groups based on their histopathological characteristics, i.e., benign (adenoma) and malignant (carcinoma), and also classified as functioning (or hormonal) and non-functioning (or non-hormonal) tumors, depending on the presence or absence of recognizable clinical syndromes due to excessive steroids. The syndrome of functioning adrenocortical tumors includes Cushing's syndrome, primary aldosteronism and the adrenorge genital syndrome, of which a minority presents most of the specific clinical features: Cushing's syndrome; red face, typical moon face, truncal obesity, and purplisch red striae, primary aldosteronism; muscle weakness, noctural polyuria, hypertension and hypokalemia, adrenogenital syndrome; virilization or feminization, but many of them present complete clinical picture. The diagnosis of these syndromes needs to measure urinary 17-OHCS and 17-KS and plasma concentrations of cortisol, aldosterone, dehydroepiandrosterone (DHEA) and the other steroids. Dexamenthasone suppression test, various stimulation tests and the measurement of plasma ACTH are also useful for diagnosis. Usually, adrenocortical tumors can be detected preoperatively by physical examination or radiographic studies. Some are massive enough to be palpable through the abdominal wall. Some are large enough to cause displacement of the kidney, as seen intravenous urography. Most are visible by adrenal scintigraphy using 131I-iodocholesterol, computerized tomography, or adrenal arteriography. The standard treatment for adrenocortical tumors are surgical resection. Unresectable adrenocortical carcinomas may be treated with an adrenocorticolytic drug, o'p'-DDD. Metyrapone and aminoglutethimide can be also employed to inhibit the production of steroids.
肾上腺皮质肿瘤可根据其组织病理学特征分为两组,即良性(腺瘤)和恶性(癌),也可根据是否存在因类固醇过多导致的可识别临床综合征,分为功能性(或激素性)和非功能性(或非激素性)肿瘤。功能性肾上腺皮质肿瘤综合征包括库欣综合征、原发性醛固酮增多症和肾上腺性征异常综合征,其中少数表现出大多数特定临床特征:库欣综合征表现为脸红、典型满月脸、躯干肥胖和紫红色条纹;原发性醛固酮增多症表现为肌无力、夜尿增多、高血压和低钾血症;肾上腺性征异常综合征表现为男性化或女性化,但许多患者呈现完整的临床表现。这些综合征的诊断需要检测尿17-羟皮质类固醇(17-OHCS)和17-酮类固醇(17-KS)以及血浆皮质醇、醛固酮、脱氢表雄酮(DHEA)和其他类固醇的浓度。地塞米松抑制试验、各种刺激试验以及血浆促肾上腺皮质激素(ACTH)的测定对诊断也有帮助。通常,肾上腺皮质肿瘤可通过体格检查或影像学检查在术前发现。有些肿瘤大到足以通过腹壁摸到。有些大到足以导致肾脏移位,如静脉肾盂造影所见。大多数肿瘤通过使用131I-碘胆固醇的肾上腺闪烁显像、计算机断层扫描或肾上腺动脉造影可见。肾上腺皮质肿瘤的标准治疗方法是手术切除。无法切除的肾上腺皮质癌可用肾上腺皮质溶解药物o,p'-DDD治疗。美替拉酮和氨鲁米特也可用于抑制类固醇的产生。