Individuals with PBMAH most commonly present with Cushing syndrome, which is characterized by sustained exposure to excess glucocorticoids. Signs and symptoms may include fatigue, central obesity, facial rounding, easy bruising, supraclavicular fat deposition, and muscle weakness, amongst others. Patients with Cushing syndrome may also have metabolic abnormalities, including hypertension, diabetes mellitus, osteoporosis, and are at an increased risk for infection. Mineralocorticoid excess, alone or in conjunction with hypercortisolemia, as well as estrogen or androgen excess are additional, rare, presentations of PBMAH. This plurihormonal characteristic of PBMAH can arise from the expression of several aberrant receptors in adrenal tissues. Disease onset typically occurs later in life, between 40 to 60 years of age, and almost never in children.
Biochemical dysregulations in PBMAH include elevated urinary free cortisol and 17-hydroxysteroids, elevated late-night serum cortisol, suppressed ACTH, and lack of suppression of serum cortisol after the dexamethasone suppression test. However, it is not uncommon for Cushing syndrome to be subclinical, and some cases are found incidentally on imaging. On abdominal imaging such as computed tomography (CT), PBMAH typically presents with large (> 1 centimeter) multilobular nodules on both adrenal glands. Typical pathology includes golden yellow-colored nodules lacking necrosis and hemorrhage. Internodular atrophy may be observed from zona reticularis atrophy.