Endocrine Abstracts

Introduction: Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH) represents <2% of all causes of Cushing’s Syndrome (CS). Clinical course is insidious, with adrenal bilateral macronodules and gradual cortisol excess, only rarely presenting with overt CS. The pathophysiology remains unclear in most cases; however, pathogenic variants in the onco-supressor ARMC5 gene are described in 25-50% of PBMAH and may confer a more severe clinical course. <p class="abs...

Introduction: Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH) represents <2% of causes of Cushing’s Syndrome (CS). Clinical course is insidious, with adrenal bilateral macronodules and gradual cortisol excess, only rarely presenting with overt CS. The pathophysiology remains unclear, however, hyperactivation of the cAMP/PKA pathway, in 77-87% of cases through aberrantly expressed G-protein coupled receptors (GPCRs), has been described. Despite being a benig...