A Case of Bilateral Macronodular Adrenocortical Hyperplasia / 대한내분비학회지

ABSTRACT

Cushing's syndrome associated with nodular adrenal glands will be divided into four main categories adrenal adenoma, adrenal carcinoma, primary pigmented nodular adrenal dysplasia (PPNAD) and macronodular adrenal hyperplasia(MAH). The term macronodular adrenal hyperplasia is restricted to the presence of multiple nodules visible to the naked eye, ranging in size from 0.5 to 7.0 cm. We report a case of Cushings syndrome caused by bilateral macronodular adrenal hyperplasia (MAH). A 45-year-old man presented with Cushingoid features, hypertension and diabetes mellitus. Urine free cortisol was 449.9 mmol/day(27-276) and were not suppressed after administration of low-dose and high-dose dexamethasone. Plasma ACTH was very low(1.87 pmol/L(18)) and was not stimulated by administration of ovine CRH. In abdominal CT, both adrenal glands were markedly enlarged and nodular in appearance. Pituitary MRI showed no abnormal finding. Bilateral adrenalectomy was done. Histologic examination revealed multiple nodules and internodular hyperplasia. This case and other reports suggested that because of variable biochemical, radiologic and pathologic findings, macronodular adrenal hyperplasia represents a heterogeneous group of patients with varying degrees of adrenal autonomy.