Introduction: The estimated annual incidence of Cushings disease ranges from 2 to 4 per million. Bilateral macronodular adrenal hyperplasia (BMAH) accounts for <1% of Cushings syndrome causes.
Case report: We present a case of 58-year-old man who had been suffering typical clinical signs of hypercortisolism for 5 years. He presented hyperglycemia, arterial hypertension, central obesity, thin skin, hemorrhagic diathesis, edemas, weakness and emotional lability. Endocrine evaluation of adrenocortical axis proved hypercortisolism of adrenal origin with increased 24-h urinary free cortisol (UFC) (792 μg/24 h [4.3176]), a low doses dexamethasone test with not suppression (21.6 μg/dl [<1.8]), a loss of cortisol circadian rhythm (16.1 and 20.6 μg/dl [<7.5]) and low ACTH concentrations (<5 pg/ml [3.719.4]). Magnetic resonance imaging of the adrenal gland demonstrated bilateral adrenal masses (the right and the left adrenal measured were 3.1×5×3 and 5×7×3 cm respectively). A NP-59 iodocholesterol scintigraphy showed an increased uptake of both adrenal glands.
With the diagnosis of Cushings syndrome ACTH-independent caused by bilateral adrenal masses surgery was made. Bilateral adrenalectomy was performed and histopathology revealed massive BMAH with multiple nodules ranging in size from several mm to 3 cm. The weight of right and left adrenal gland was 85 and 105 g respectively.
After that, hypercortisolism was solved and patients phenotype, diabetes and hypertension disappeared.
Conclusions: BMAH is rare cause of Cushings syndrome. It more often presents as an incidental radiological finding with underlying subclinical hypercortisolism. It is usually detected in the fifth or sixth decade of life. Bilateral adrenalectomy remains the main treatment for BMAH but unilateral adrenalectomy has been proposed in selected cases.