Adrenocortical tumor in a patient with untreated congenital adrenocortical hyperplasia owing to 21-hydroxylase deficiency: characterization of steroidogenic lesions

Abstract We report a case of congenital adrenal hyperplasia complicated by a right adrenal adenoma. The congenital adrenal hyperplasia was confirmed by extremely high levels of basal serum 17«-hydroxy-progesterone and urinary pregnantriol as well as an exaggerated response of lYce-hydroxyprogester-one to adrenocorticotropic hormone. On roentogenographic examinations and an echogram a huge calcified tumor was seen in the right adrenal gland. Histological findings indicated an adrenocortical adenoma. Culture of the tumor cells showed an accentuated response in the ratio of 17«-hydroxy-progesterone to desoxycortisol with adrenocorticotropic hormone as a stimulator, suggesting that such adenoma cells also lack 21-hydroxylase activity. Our study clearly demonstrates that tumor cells have the same steroidogenic lesions as hyperplasia.