异位促肾上腺皮质激素综合征的诊断治疗进展

Progress of the Diagnosis and Treatment of the Ectopic Adrenocorticotropic Hormone Syndrome

异位促肾上腺皮质激素综合征(EAS)由不同的内分泌或非内分泌肿瘤分泌促肾上腺皮质激素(ACTH)导致肾上腺皮质增生并分泌过量的皮质醇引起的一种临床综合征。联合不同的试验和影像学检查对准确诊断异位ACTH十分关键。如能早期发现异位肿瘤来源并行根治性切除,一般预后较好。对于未能发现异位肿瘤来源或者没有手术适应证的患者,通过药物控制高皮质醇血症,可以明显降低患者的并发症,提高患者的生活质量,延长患者的生命。因此单纯药物治疗在某种程度上可以代替双侧肾上腺切除手术。

Ectopic adrenocorticotrepie hormone syndrome（EAS） is a clinical syndrome caused by the secretion of adrenalcortical hormone（ACTH） in adrenalcortex hyperplasia and secretion of excess cortisol caused by different endocrine and non-endocrine tumors. A variety of tests and imaging studies are necessa- ry for the correct diagnosis of the EAS. The first-line treatment of EAS is surgery. However,chemotherapy is necessary when surgery is not successful, not indicated, or failed to detect source of EAS. Cortisol-lowering chemotherapy can significantly reduce complications, improve the quality of life of patients and prolong the life of patients. So simple medical treatment has a good effect, might be an alternative to bilateral adrenalectomy.