双侧肾上腺占位致ACTH非依赖性库欣综合征(附1例报告及文献复习)

Bilateral adrenal masses with ACTH-independent Cushing syndrome(with a report of one new case and related literature review)

目的探讨双侧肾上腺占位致ACTH非依赖性库欣综合征的临床特征。方法回顾性分析北京协和医院1例及PUBMED检索到的21例、中国知网检索到的1例双侧肾上腺占位致ACTH非依赖性库欣综合征的临床资料。结果 23例患者的临床特征是:(1)发病年龄46.9±13.8岁。(2)双侧肾上腺占位多见于女性,23例病人中仅有4例为男性。(3)双侧肾上腺占位致ACTH非依赖性库欣综合征可以有典型库欣综合征(CS)表现,也可以仅表现为亚临床库欣(SCS)(16例SCS,7例CS)。(4)双侧肾上腺占位的大小、平均重量、病理特征和功能状态可以不一致。结论成功的肾上腺静脉取血可以帮助判断双侧占位的功能状态,据此做出恰当的外科处理,部分患者术后需激素替代治疗。

Objective To investigate the clinical characters of bilateral adrenal masses with ACTH-independent cushing syndrome. Methods The clinical data of one new case from the PUMC Hospital, twenty-one cases from PUBMED and one case from CNKI were retrospectively analyzed. Results The following clinical characters of the twenty-three cases were discovered： （1） the age of onset was at 46.9^-13.8 years old; （2） BAM occurs in women more than in men （19 women, 4 men in 23 patients）; （3） Six of the 23 patients had clinically overt cushing syndrome, and the remainder had subclinical cushing syndrome; （4） The size, weight, pathology and function of BAM could not be identical. Conclusions Successful AVS is very helpful to definite the function of BAM and can help determine appropriate management. Glucocorticoid replacement is required for months after surger in some patients.