Abstract
ObjectivePrimary 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.DesignWe performed a retrospective analysis of 16 patients with PBMAH and clinical overt cortisol secretion in three centersMethodsSelectivity 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.ResultsAVS 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.ConclusionsIn this study the gain of information through AVS for unilateral adrenalectomy was limited in patients with PBMAH and marked adrenal asymmetry.
Highlights
Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing’s syndrome. It is usually identified by adrenal imaging demonstrating bilateral enlarged adrenal masses [1] composed of multiple bilateral macronodules (>10 mm) with hyperplasia and/or internodular atrophy
Several studies indicated the effectiveness of unilateral adrenalectomy in bilateral disease [6–11]
Our group has analyzed the long-term outcome after unilateral adrenalectomy for PBMAH and showed that unilateral adrenalectomy leads to clinical remission and a lower incidence of adrenal crisis [12]
Summary
Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing’s syndrome. It is usually identified by adrenal imaging demonstrating bilateral enlarged adrenal masses (symmetric and asymmetric) [1] composed of multiple bilateral macronodules (>10 mm) with hyperplasia and/or internodular atrophy. According to the guidelines of the Endocrine Society, firstline treatment of PBMAH with overt Cushing’s syndrome should be bilateral adrenalectomy with consecutive remission and lifelong requirement for steroid replacement therapy [5]. It appeared from this publication that some patients may have persistent hypercortisolism to a clinically relevant degree and potentially higher mortality.
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