YOUNG FEMALE-PATIENT WITH TESTOSTERONE-PRODUCING ADRENOCORTICAL ADENOMA ALSO SHOWING SIGNS OF SUBCLINICAL CUSHINGS-SYNDROME

A 28-year old female patient with virilization due to left adrenocorti cal adenoma was studied. The patient had clinical features of hyperand rogenism such as hirsutism and a low pitched voice, but not of hyperco rticoidism. Plasma testosterone and dehydroepiandrosterone-sulfate (DH EA-S) were high. Although the basal plasma cortisol concentration and urinary excretion of 17-hydroxycorticosteroids (17-OHCS) were within t he normal range, the absence of diurnal variation in plasma cortisol a nd loss of suppressibility by dexamethasone suggested constitutive sec retion of cortisol by the tumor. Inappropriate cortisol secretion was also supported by blunted ACTH response to provocative stimuli. After successful removal of the left adrenal tumor, such endocrinological ab normalities were all normalized. Immunohistochemical analysis revealed that tumor cells were positively stained for C21 hydroxylase cytochro me P-450 (P-450(C21)) and P-450(11 beta) which convert 17-hydroxy (OH) progesterone to cortisol as well as P-(SCC)-S-450, 3 beta-hydroxyster oid dehydrogenase and P-450(17 alpha) which are involved in testostero ne biosynthesis. These findings suggest that adrenocortical adenoma se cretes predominantly testosterone and constitutively cortisol in a you ng woman patient with virilization.