BILATERAL MASSIVE MACRONODULAR ADRENAL-HY PERPLASIA, A RARE CAUSE OF CUSHINGS-SYNDROME

A 46-year-old man with known arterial hypertension for 10 years had, o ver the last two years, developed increasing obesity, particularly of the trunk, with other symptoms typical of Cushing's syndrome. Hormone analysis demonstrated hypercortisolism and decreased plasma ACTH conce ntration. The dexamethasone inhibition test failed to show any signifi cant suppression of serum cortisol. Plasma ACTH was not increased in t he corticotrophin-releasing hormone and the metyrapone tests. In the s hort ACTH test there was an excessive cortisol increase. Abdominal com puted tomography revealed both adrenals to be enlarged (6 x 4 cm) and coarsely nodular. Adrenolytic treatment with ketoconazole (400 mg dail y) caused symptoms of adrenal insufficiency, but a reduced dosage of 2 00 mg daily lowered the cortisol level to between 5 and 11 mu g/dl and normalized the blood pressure and clinical signs of Cushing's syndrom e disappeared. Subsequent bilateral adrenalectomy confirmed the diagno sis of massive macronodular adrenal hyperplasia. Substitution treatmen t with twice daily 25 mg cortisone acetate and 0.05 mg fludrocortisone was started postoperatively.