ABSTRACT
@EN ACTH-dependent bilateral adrenocortical hyperplasia, known as Cushing's disease, accounts for 60-70% of the cases of Cushing's syndrome. In about 30-40% of patients, manifestations of Cushing's syndrome occur independently of pituitary ACTH
secretion due to either ectopic ACTH production or an adrenocortical adenoma or carcinoma. Another rere cause of Cushing's syndrome, also independent of ACTH, is so-called primary adrenocortical nodular dysplasia, in which adjacent areas of the
adrenal
cortex are atropic. The term bilateral macronodular adrenocortical hyperplasia (MNH) is restricted to the presence of multiple nodules visible to the naked eye and often more than 0.4cm in diameter. Its pathogenesis is still unknown. This report
describes a patient with Cushing's syndrome due to ACTH-independent bilateral macronodular adrenocortical hyperplasia associated empty sella syndrome. A 63-year-old woman presented with Cushingoid features, hypertension, diabetes mellitus,
dyspnea
and
chest pan. Urinary free cortisol and 17-hydroxycorticosteroi excretion were 350ug/day and 10.9mg/day, respectively. Urinary free cortisol didn't decrease after administration of dexamethasone (low and high dose). Plasma ACTH was 4.2pg/ml.
Magnetic
resonance imaging (MRI) of the sella turcica revealed complete cisternal hernation, suggesting empty sells syndrome. Bilateral total adrenalectomy was perfomed. The right adrenal gland weighed 11 gm and contained two nodules and their sizes are
2.5cm,
and 1cm. The left adrenal gland showed all the features of nodular hyperplasia with at 1.5cm nodule. The postoperative course was uneventful and the patient was discharged to be treated with replacement steroids. (J Kor Soc Endocrinol 8:203~210,
1993)
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