促肾上腺皮质激素非依赖性库欣综合征4例临床分析并文献复习

Clinical analysis and literature review of 4 cases of adrenocorticotropic hormone-independent Cushing's syndrome

目的探究不同病因导致促肾上腺皮质激素(ACTH)非依赖性库欣综合征的临床特点及治疗。方法结合有关文献对4例ACTH非依赖性库欣综合征患者的临床资料进行回顾性分析。结果4例患者均为女性,年龄24~56岁,有库欣综合征的症状及体征,实验室检查符合ACTH非依赖性库欣综合征。2例患者行单侧肾上腺切除术,术后病理提示肾上腺皮质腺瘤、原发性色素沉着性结节性肾上腺皮质病(PPNAD);1例患者行左侧肾上腺切除术后病理诊断为ACTH非依赖性大结节增生(AIMAH),术后4+年复查皮质醇高,再次行右侧肾上腺大部切除术;另1例患者考虑诊断为肾上腺皮质癌,因合并肝脏、肺部多发转移,间断口服米托坦治疗。结论不同病因导致ACTH库欣综合征患者的临床表现、实验室检查相似,治疗方法却不同,应加强对该类疾病的认识,旨在提高诊治水平。

Objective To explore the clinical characteristics and treatment of adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome with different causes.Methods The clinical data of 4 cases of-independent Cushing's syndrome was retrospectively analyzed with reference to the relevant literature.Results All 4 patients were female,aged 24-56 years,with symptoms and signs of Cushing's syndrome,and laboratory tests were consistent with ACTH non-dependent Cushing's syndrome.2 patients had undergone unilateral adrenalectomy,with the postoperative pathology revealing adrenocortical adenoma and primary pigmented nodular adrenocortical disease(PPNAD).1 patient had undergone left adrenalectomy and was pathologically diagnosed with ACTH-independent macronodular adrenal hyperplasia(AIMAH);4+years after the surgery,the patient was noted with elevated cortisol and subtotal right adrenalectomy was performed.1 patient had been considered with the diagnosis of adrenocortical carcinoma,and was treated with intermittent oral mitotane for the associated liver and lung metastases.Conclusion For patients with Cushing's syndrome of different causes,the clinical manifestations and laboratory examination results are similar,yet the treatment regimens varies.Further understanding of the disease is warranted to improve its diagnosis and treatment.