Cushing's Syndrome Associated With an Adrenal Neuroblastoma

Abstract

CUSHING'S SYNDROME is uncommon in childhood and rare in infancy. In these age groups it is usually due to adrenal cortical tumors, although cases of adrenal hyperplasia are recorded. Approximately 60 adults have been reported, in whom Cushing's syndrome developed in association with nonendocrine tumors, ie, tumors not of pituitary, adrenocortical, or gonadal origin. Our patient had an adrenal neuroblastoma and was similar to the majority of these adults, in that sudden onset of symptoms, marked weakness associated with hypokalemia, and a rapidly fatal outcome characterized his course. This is the second report of Cushing's syndrome in an infant in association with a neuroblastoma. It is possible that awareness of the salient features of these cases might permit earlier diagnosis, and possibly complete tumor removal. Methods A modification of the method of Callow et al was used to measure 17-ketosteroids,¹ and 17-hydroxycorticosteroids were measured by a modification of the