An adrenocortical tumor secreting weak mineralocorticoids.
An adrenocortical carcinoma (15.5 g) secreting excessive amounts of steroids with weak mineralocorticoid activity in a 25-year-old woman was studied with particular reference to its in vivo and in vitro secretions of steroids. Severe hypertension, occasional low serum potassium and suppressed PRA were the major clinical findings, and were improved with removal of the tumor. In the preoperative stage, plasma levels of 11-deoxycorticosterone, 18-hydroxy-11-deoxycorticosterone, corticosterone and 18-hydroxycorticosterone were all increased. However, the plasma level of aldosterone was repeatedly normal. Although plasma levels of pregnenolone, 17-hydroxypregnenolone, progesterone and 17-hydroxyprogesterone were very high, those of other late step steroids, i.e. 11-deoxycortisol, cortisol, dehydroepiandrosterone, androstenedione and testosterone were almost normal. From these findings, a major etiological role of weak mineralocorticoids such as 11-deoxycorticosterone, 18-hydroxycorticosterone and corticosterone in her hypertension was suggested. Pregnenolone and 17-hydroxypregnenolone in tumor tissue were increased, but 11-deoxycorticosterone, corticosterone, aldosterone, cortisol and adrenal androgens such as dehydroepiandrosterone, androstenedione and testosterone were below normal or low normal. In vitro production of 11-deoxycorticosterone, aldosterone or cortisol by the tumor tissue slices was very low and scarcely responded to synthetic ACTH.
D006907:17-alpha-Hydroxypregnenolone / Q000378:metabolism; D019326:17-alpha-Hydroxyprogesterone; D015069:18-Hydroxycorticosterone / Q000378:metabolism; D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D000328:Adult; D002277:Carcinoma / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D003345:Corticosterone / Q000378:metabolism; D003900:Desoxycorticosterone / Q000031:analogs & derivatives / Q000378:metabolism; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000378:metabolism; D006973:Hypertension / Q000209:etiology; D008901:Mineralocorticoids / Q000378:metabolism*; D011284:Pregnenolone / Q000378:metabolism; D011374:Progesterone / Q000378:metabolism
D006908:Hydroxyprogesterones; D008901:Mineralocorticoids; C042509:18-hydroxydeoxycorticosterone; D006907:17-alpha-Hydroxypregnenolone; D003900:Desoxycorticosterone; D011374:Progesterone; D015069:18-Hydroxycorticosterone; D019326:17-alpha-Hydroxyprogesterone; D011284:Pregnenolone; D003345:Corticosterone