Section of Endocrinology, Department of Internal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, West Virginia 25701, USA.
Endocr Pract. 2013 May-Jun;19(3):515-21. doi: 10.4158/EP12277.RA.
To review the challenges encountered in the diagnostic work-up and management of patients with subclinical Cushing's syndrome (SCS) and bilateral adrenal masses to aid in the case description of a patient with SCS and adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH).
We describe our experience managing a patient with AIMAH and SCS. This case report is followed by an extensive review of the literature regarding differential diagnoses, work-up including adrenal venous sampling (AVS), and treatment of SCS with bilateral adrenal masses.
A 51-year-old female who was diagnosed with recent onset hypertension and diabetes mellitus type 2 was evaluated for adrenal masses discovered incidentally on computed tomography (CT). She did not have any Cushingoid features. Magnetic resonance imaging (MRI) of abdomen was performed for further evaluation. Hormonal evaluation came back consistent with SCS. The AVS results were consistent with bilateral autonomous cortisol hypersecretion without lateralization. Collectively, the findings favored the diagnosis of bilateral AIMAH. A left adrenalectomy was performed, and the patient's clinical response was favorable with improvement in blood pressure (BP) accompanied by significant weight loss. Follow-up hormonal testing for autonomous cortisol hypersecretion was within the target range.
AIMAH is a rare cause of SCS. AVS is a useful diagnostic tool that helps localize the source of autonomous cortisol hypersecretion in ACTH-independent SCS with bilateral adrenal masses, especially if radiological features are inconclusive. Patients undergoing unilateral adrenalectomy should be followed for monitoring of clinical response, as well as progression of AIMAH in the contralateral adrenal gland.
回顾在亚临床库欣综合征（SCS）和双侧肾上腺肿块患者的诊断和治疗中遇到的挑战，以辅助 SCS 和促肾上腺皮质激素（ACTH）非依赖性大结节性肾上腺增生（AIMAH）患者的病例描述。
我们描述了我们管理一位 AIMAH 和 SCS 患者的经验。该病例报告之后是对有关鉴别诊断、包括肾上腺静脉采样（AVS）在内的检查以及双侧肾上腺肿块的 SCS 治疗的文献进行了广泛的回顾。
一位 51 岁的女性因近期发现的高血压和 2 型糖尿病而就诊，其在计算机断层扫描（CT）上偶然发现了肾上腺肿块。她没有库欣样特征。为了进一步评估，对腹部进行了磁共振成像（MRI）检查。激素评估结果符合 SCS。AVS 结果符合双侧自主皮质醇分泌过多而无侧化。综合来看，这些发现支持双侧 AIMAH 的诊断。进行了左肾上腺切除术，患者的临床反应良好，血压（BP）改善，体重明显减轻。自主皮质醇分泌过多的后续激素检测在目标范围内。
AIMAH 是 SCS 的罕见病因。AVS 是一种有用的诊断工具，可帮助确定 ACTH 非依赖性 SCS 伴双侧肾上腺肿块中自主皮质醇分泌过多的来源，尤其是在影像学特征不确定的情况下。接受单侧肾上腺切除术的患者应进行临床反应和对侧肾上腺中 AIMAH 进展的监测。
