Zeiger M A, Nieman L K, Cutler G B, Chrousos G P, Doppman J L, Travis W D, Norton J A
Surgical Metabolism Section, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892.
Surgery. 1991 Dec;110(6):1106-15.
Nontumorous primary adrenal causes of Cushing's syndrome are exceedingly rare. Herein we review our results with seven patients in whom there is biochemical evidence of a primary (adrenocorticotropin independent) bilateral adrenal cause of endogenous hypercortisolism. Each patient had low plasma adrenocorticotropin levels. All patients had elevated 24-hour urinary free cortisol levels and 17-hydroxycorticosteroids that were not suppressed by high-dose dexamethasone. Plasma levels of adrenocorticotropin and cortisol were not elevated by ovine corticotropin-releasing factor. No patient had a gradient between petrosal and peripheral adrenocorticotropin levels. No pituitary tumors were detected by magnetic resonance imaging or computed tomography. Five of six patients who underwent iodocholesterol scanning showed bilateral adrenal activity. Computed tomographic and magnetic resonance imaging of the abdomen demonstrated bilateral small adrenal glands in three patients, an adrenal mass in one patient with Carney's complex, and massively enlarged glands in three patients. Each patient underwent bilateral adrenalectomy and was given glucocorticoid and mineralocorticoid replacement. Pathologic examination of four of these bilateral adrenal specimens revealed primary pigmented micronodular adrenocortical disease, with adrenal gland weights between 2.5 and 13.4 gm (mean 5.2 gm). However, the remaining three patients had primary adrenocorticotropin-independent bilateral macronodular adrenocortical disease with adrenal gland weights between 32 and 81 gm (mean 52 gm). Although each of the patients with primary pigmented micronodular adrenocortical disease was cured by bilateral adrenalectomy through a posterior approach, two of the three patients required an anterior approach. We conclude that Cushing's syndrome can arise through two distinct forms of primary bilateral adrenal cortical disease. Computed tomography is important in evaluation of these patients because the size of the adrenal glands influences the surgical approach.
库欣综合征的非肿瘤性原发性肾上腺病因极为罕见。在此，我们回顾了7例患者的情况，这些患者有生化证据表明存在原发性（促肾上腺皮质激素非依赖性）双侧肾上腺导致的内源性皮质醇增多症。每位患者的血浆促肾上腺皮质激素水平均较低。所有患者的24小时尿游离皮质醇水平和17-羟皮质类固醇水平均升高，且不受高剂量地塞米松抑制。羊促肾上腺皮质激素释放因子未使血浆促肾上腺皮质激素和皮质醇水平升高。没有患者岩下窦和外周促肾上腺皮质激素水平存在梯度差异。磁共振成像或计算机断层扫描未检测到垂体肿瘤。6例接受碘胆固醇扫描的患者中有5例显示双侧肾上腺有活性。腹部计算机断层扫描和磁共振成像显示，3例患者双侧肾上腺较小，1例患有卡尼综合征的患者有肾上腺肿块，3例患者肾上腺明显增大。每位患者均接受了双侧肾上腺切除术，并给予糖皮质激素和盐皮质激素替代治疗。对其中4例双侧肾上腺标本进行病理检查发现为原发性色素沉着性微结节性肾上腺皮质疾病，肾上腺重量在2.5至13.4克之间（平均5.2克）。然而，其余3例患者患有原发性促肾上腺皮质激素非依赖性双侧大结节性肾上腺皮质疾病，肾上腺重量在32至81克之间（平均52克）。虽然原发性色素沉着性微结节性肾上腺皮质疾病的每位患者通过后路双侧肾上腺切除术均治愈，但3例患者中有2例需要前路手术。我们得出结论，库欣综合征可通过两种不同形式的原发性双侧肾上腺皮质疾病发生。计算机断层扫描在评估这些患者时很重要，因为肾上腺的大小会影响手术方式。
