Primary Adrenocortical Nodular Dysplasia: A Rare Cause of Cushing's Syndrome1

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Abstract
Pituitary-adrenal function was studied before and after bilateral adrenalectomy in a 14-yr-old girl with an unusual form of Cushing's syndrome. Manipulative tests prior to surgery revealed urinary 17-hydroxycorticosteroids (17-OHCS) which were unsuppressible with dexamethasone (2 and 8 mg/day), unresponsive to exogenous ACTH, and which fell to very low levels with metyrapone administration. A small sella turcica (lateral area 47 mm2) was demonstrated. Both adrenals demonstrated multiple small nodules and sheets of atypical tissue surrounded by more normal appearing adrenocortical cells. Plasma ACTH was undetectable even at low levels (<.12 mU/100 ml) during the surgical procedure and for 2 months after adrenalectomy even during periods of complete withdrawal of cortisol therapy. Plasma ACTH returned to a normal but not elevated level 3 and 7 months after adrenalectomy while the patient was receiving 30 mg of cortisol/day. The results of these studies indicate that the bilateral adrenal hyperfunction was non-ACTH dependent and that pituitary ACTH secretion was profoundly suppressed. Comparative studies of plasma ACTH levels in patients with other forms of Cushing's syndrome and the sum of all evidence indicate that the lesion in this patient was primary in the adrenal cortex. The term “primary adrenocortical nodular dysplasia” is suggested to describe this entity and to distinguish it from the more common forms of ACTH dependent bilateral hyperplasia.