双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征3例临床诊疗探索

目的 探讨双侧肾上腺高功能皮质腺瘤致促肾上腺皮质激素（ACTH）非依赖性库欣综合征的临床特点、诊治思路，提高对该疾病的认知。 方法 收集2016～2021年四川大学华西医院内分泌代谢科收治的3例双侧肾上腺功能性皮质腺瘤致ACTH非依赖性库欣综合征患者行回顾性分析，分析患者的一般情况、症状及体征、辅助检查、诊治过程等临床资料，并结合文献进行复习和讨论。 结果 3例患者均表现为典型且严重的库欣综合征，并伴有显著的心脏和代谢并发症，影像学提示双侧肾上腺单发腺瘤；2例患者行双侧肾上腺静脉采血，均提示双侧肾上腺皮质醇高分泌且无优势侧；1例患者行双侧肾上腺肿物切除术，另外2例患者因为心功能不全严重，行分期双侧肾上腺肿物切除术，术后病理结果3例均提示双侧肾上腺皮质腺瘤，术后皮质醇浓度均明显降低，均给长期予糖激素替代治疗。 结论 双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征非常罕见，明确诊断并判断双侧功能定位非常重要，治疗上应根据患者的病情及并发症情况个体化的选择最适合患者的手术方式，术后应严密随访，及时给予并调整糖皮质激素替代治疗，并终身随访。

Clinical diagnosis and treatment of ACTH-independent Cushing's syndrome caused by bilateral adrenal functional cortical adenoma: a report of 3 cases

Objective To analyze the clinical characteristics, diagnosis and treatment of ACTH-independent Cushing''s syndrome caused by bilateral adrenal high functional cortical adenomas, and improve the cognition of the disease. Methods This paper reports the general situation, symptoms and signs, auxiliary examination, diagnosis and treatment of 3 patients with ACTH-independent Cushing''s syndrome caused by bilateral adrenal functional cortical adenomas in Adrenal Center, the Department of Endocrinology and Metabolism, West China Hospital. Results All 3 patients showed typical and severe Cushing''s syndrome, and severe cardiac and metabolic complications. CT scan imaging showed bilateral adrenal solitary nodules. Two patients underwent bilateral adrenal veins sampling, which indicated that both high secretion of bilateral adrenal gland. One patient underwent simultaneous bilateral adrenalectomy, and the other 2 patients underwent bilateral adrenalectomy by stages because of serious cardiac insufficiency. Postoperative pathology showed bilateral adrenocortical adenoma in 3 cases, and the cortisol concentration decreased significantly after operation. All patients were given long term glucocorticoid replacement therapy after operation. Conclusion ACTH-independent Cushing''s syndrome caused by bilateral adrenal functional cortical adenoma is very rare, so it is very important to make a clear diagnosis and judge the bilateral functional orientation. In treatment, the most suitable surgical method should be selected individually according to the patient''s condition and complications. After operation, close follow up should be made, glucocorticoid replacement therapy should be administrated and adjusted in time, and lifelong follow-up.