Tsigos C, Chrousos G P
Department of Experimental Physiology, University of Athens Medical School, Greece.
Annu Rev Med. 1996;47:443-61. doi: 10.1146/annurev.med.47.1.443.
The diagnosis of endogenous Cushing's syndrome requires demonstration of an increased cortisol secretion rate, best achieved by urinary free cortisol excretion determinations. In borderline or confusing cases, loss of diurnal cortisol rhythmicity, a combined dexamethasone/corticotropin releasing hormone (CRH) test, or close monitoring of the patient for a few months will be helpful in ruling out pseudo-Cushing's. Primary adrenal Cushing's syndrome can be ruled out on the basis of a normal or elevated basal and/or CRH-stimulated plasma adrenocorticotropin (ACTH) and a negative adrenal computed tomography. ACTH-dependent Cushing's syndrome can then be differentiated on the basis of a CRH test and imaging procedures. A discrete pituitary lesion on magnetic resonance imaging and a standard CRH test with results consistent with such a lesion are sufficient to proceed to transsphenoidal surgery. If no discrete pituitary lesion is present, or if the CRH test is equivocal, bilateral simultaneous inferior petrosal sinus sampling with CRH administration is necessary to distinguish between a pituitary and an ectopic source. Surgery is the treatment of choice for all types of Cushing's syndrome. In the few cases in which transsphenoidal surgery fails or the disease recurs, repeat transsphenoidal surgery, or radiation therapy in association with mitotane treatment, is a reasonable alternative. Bilateral adrenalectomy effectively cures hypercortisolism if resection of the ACTH-secreting tumor is unsuccessful and radiation/medical therapy fails.
内源性库欣综合征的诊断需要证明皮质醇分泌率增加，最好通过测定尿游离皮质醇排泄量来实现。在临界或疑难病例中，皮质醇昼夜节律消失、地塞米松/促肾上腺皮质激素释放激素（CRH）联合试验或对患者进行数月的密切监测有助于排除假性库欣综合征。原发性肾上腺皮质库欣综合征可根据基础和/或CRH刺激的血浆促肾上腺皮质激素（ACTH）正常或升高以及肾上腺计算机断层扫描阴性来排除。然后可根据CRH试验和影像学检查来区分ACTH依赖性库欣综合征。磁共振成像上有明确的垂体病变以及标准CRH试验结果与此类病变相符，就足以进行经蝶窦手术。如果没有明确的垂体病变，或者CRH试验结果不明确，则需要在注射CRH的情况下进行双侧同时岩下窦采血，以区分垂体来源和异位来源。手术是所有类型库欣综合征的首选治疗方法。在少数经蝶窦手术失败或疾病复发的病例中，重复经蝶窦手术或联合米托坦治疗的放射治疗是合理的替代方案。如果切除分泌ACTH的肿瘤不成功且放射/药物治疗失败，双侧肾上腺切除术可有效治愈皮质醇增多症。
