Virilizing adrenocortical oncocytoma in a toddler

Abstract Functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40–60 yr of age. To our knowledge, only 6 cases of functioning adrenocortical oncocytomas have been reported in childhood. We report a case of functioning adrenocortical oncocytoma in a 3 year 4 month-old female child presenting with hirsutism, acne and virilization. She presented with coarse features, deepening of the voice and excessive hair growth, and elevation of plasma testosterone and dehydroepiandrosterone sulfate. She had an adrenalectomy. The completely resected tumor composed predominantly of oncocytes without atypical mitosis and necrosis. A discussion of this case and a review of the literature on this entity are presented.