Primary adrenocortical nodular dysplasia as a cause of Cushing's syndrome in infants and children

Abstract

Two patients with Cushing's syndrome due to primary adrenocortical nodular dysplasia (PAND) are described. Both patients, an infant and a 14-year-old boy at the time of diagnosis, were treated by total bilateral adrenalectomy. On gross inspection, the adrenal glands of the infant did not appear to be abnormal but those of the older patient showed numerous dark nodules. Microscopically, the adrenal glands of each patient manifested multiple micronodules with internodular atrophy. The extreme internodular atrophy suggest that primary adrenocortical nodular dysplasia is a non-ACTH-dependent condition. Since the disorder appears to involve primarily the cortex of both adrenals, adrenalectomy followed by steroid replacement is the recommended treatment.