An Unusual Variant of Cushing Syndrome

ObjectiveTo discuss the initial clinical manifestations of primary pigmented nodular adrenocortical disease.MethodsWe present a case report of a 4-year-old boy who had the classic clinical features of Cushing syndrome. Results of hormonal investigations are reviewed, and histopathologic findings are illustrated.ResultsInvestigations revealed adrenocorticotropic hormone (corticotropin)-independent Cushing syndrome. Findings on magnetic resonance imaging of the pituitary gland and abdomen were within normal limits. The patient underwent bilateral adrenalectomy. The histopathologic features were consistent with primary pigmented nodular adrenocortical disease.ConclusionPrimary pigmented nodular adrenocortical disease should be suspected in patients with corticotropin- independent Cushing syndrome who have normal findings on adrenal imaging. (Endocr Pract. 2008;14:717-720)