Challenges in Diagnosis and Treatment of Cushing Syndrome in Bilateral Macronodular Adrenal Hyperplasia

Lietuvos Chirurgija Pub Date : 2022-04-27 DOI:10.15388/lietchirur.2022.21.57

Edvina Januškevičiūtė, Laura Kalakauskaitė, Ž. Visockienė

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Abstract

Background. Bilateral adrenal cortical hyperplasia (ACH) is one of the rare causes of adrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome (CS), where lateralization of cortisol secretion and choice of treatment techniques are challenging. Percutaneous radiofrequency ablation (RFA) is a safe and effective minimally invasive treatment for benign and malignant tumors, but it is not commonly used to treat CS in bilateral ACH. Case description. A 79-year-old patient developed ACTH-independent CS with bilateral ACH. Adrenal venous sampling (AVS) showed right sided cortisol hypersecretion. Due to serious comorbidities it was decided to perform percutaneus RFA. The procedure was successful and without any complications. Hydrocortisone replacement therapy was given to manage adrenal insufficiency after the procedure. Conclusions. Percutaneous RFA is an effective minimally invasive procedure for the treatment of cortisol producing adrenal tumors. Reduction of symptoms caused by the disease has been observed after the procedure. It is also expected that hormone deficiency developed after the procedure will be transient.

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双侧肾上腺大结节性增生库欣综合征的诊治挑战

背景双侧肾上腺皮质增生（ACH）是促肾上腺皮质激素（ACTH）非依赖性库欣综合征（CS）的罕见原因之一，皮质醇分泌的侧化和治疗技术的选择具有挑战性。经皮射频消融术（RFA）是一种安全有效的良恶性肿瘤微创治疗方法，但在治疗双侧ACH的CS中并不常用。案例描述。一名79岁的患者发展为ACTH非依赖性CS伴双侧ACH。肾上腺静脉取样（AVS）显示右侧皮质醇分泌过多。由于严重的合并症，决定进行经皮射频消融。手术成功，没有任何并发症。术后应用氢化可的松替代治疗肾上腺功能不全。结论。经皮RFA是一种有效的微创手术，用于治疗产生皮质醇的肾上腺肿瘤。手术后观察到由该疾病引起的症状减轻。手术后出现的激素缺乏也是暂时性的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Lietuvos Chirurgija

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24 weeks