Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, München, Germany.
Department of Internal Medicine I, Division of Endocrinology and Diabetes, University of Würzburg, University Hospital Würzburg, Würzburg, Germany.
Endocrine. 2022 May;76(2):434-445. doi: 10.1007/s12020-022-03020-z. Epub 2022 Mar 10.
Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing's syndrome. Current guidelines recommend bilateral adrenalectomy for PBMAH, but several studies showed clinical effectiveness of unilateral adrenalectomy despite bilateral disease in selected patients. Our aim was to evaluate the gain of information which can be obtained through adrenal venous sampling (AVS) based cortisol lateralization ratios for guidance of unilateral adrenalectomy.
We performed a retrospective analysis of 16 patients with PBMAH and clinical overt cortisol secretion in three centers METHODS: Selectivity of adrenal vein sampling during AVS was defined as a gradient of cortisol or a reference adrenal hormone ≥2.0 between adrenal and peripheral vein. Lateralization was assumed if the dominant to non-dominant ratio of cortisol to reference hormone was ≥4.0.
AVS was technically successful in all patients based on absolute cortisol levels and in 13 of 16 patients (81%) based on reference hormone levels. Lateralization was documented in 8 of 16 patients. In patients with lateralization, in 5 of 8 cases this occurred toward morphologically larger adrenals, while in 3 patients lateralization was present in bilaterally identical adrenals. The combined volume of adrenals correlated positively with urinary free cortisol, suggesting that adrenal size is the dominant determinant of cortisol secretion.
In this study the gain of information through AVS for unilateral adrenalectomy was limited in patients with PBMAH and marked adrenal asymmetry.
双侧大结节性肾上腺皮质增生症（PBMAH）是促肾上腺皮质激素（ACTH）非依赖性库欣综合征的罕见病因。目前的指南建议对 PBMAH 行双侧肾上腺切除术，但多项研究表明，在某些选定的患者中，尽管双侧存在疾病，单侧肾上腺切除术也具有临床疗效。我们的目的是评估通过肾上腺静脉取样（AVS）获得的皮质醇侧化比值信息，以指导单侧肾上腺切除术。
我们在三个中心对 16 例 PBMAH 患者和临床表现为皮质醇过度分泌的患者进行了回顾性分析。
AVS 时肾上腺静脉的选择性定义为皮质醇或参考肾上腺激素的梯度≥2.0。如果优势肾上腺与非优势肾上腺的皮质醇与参考激素的比值≥4.0，则认为存在侧化。
根据绝对皮质醇水平，所有患者的 AVS 均在技术上取得成功，根据参考激素水平，16 例患者中有 13 例（81%）取得成功。16 例患者中有 8 例存在侧化。在存在侧化的患者中，5 例发生在形态上较大的肾上腺，而在 3 例患者中，侧化发生在双侧相同大小的肾上腺。肾上腺的总体积与尿游离皮质醇呈正相关，表明肾上腺大小是皮质醇分泌的主要决定因素。
在这项研究中，对于双侧肾上腺存在明显不对称的 PBMAH 患者，通过 AVS 获得的单侧肾上腺切除术的信息增益有限。
