We report a case of
hypertension,
hypokalemia, and
amenorrhea accompanying an
adrenocortical carcinoma. A 27-year-old woman was admitted to our hospital because of a left
adrenal incidentaloma. She presented with
hypertension,
hypokalemia, and
amenorrhea; her plasma
renin activity was low, but her plasma
aldosterone concentration was normal, as were
cortisol and
androgens. By contrast, her serum concentrations of
deoxycorticosterone (DOC),
18-hydroxydeoxycorticosterone, and
progesterone were high, and her urinary
steroid profile showed elevated secretion of 17-deoxysteroids and 11-deoxysteroids (
progesterone, DOC, 11-dehydrocorticosterone, and
11-deoxycortisol), and 3beta-hydroxy 5-en
steroids (
pregnenolone,
17-hydroxypregnenolone, and
DHEA). Decreased ratios of metabolites of (1) 17-OHpregnenolone to
pregnenolone and 17-OHprogesterone to
progesterone, (2)
corticosterone to DOC and
cortisol to
11-deoxycortisol, and (3)
progesterone to
pregnenolone, 17-OHprogesterone to 17-OHpregnenolone and
androstenedione to
DHEA suggested the impairment of 17alpha-hydroxylase, 11beta-hydroxylase, and 3beta-HSD activities, respectively. After the
tumor was removed, levels of all adrenal
steroids were normalized. Based on the Weiss criteria, the
tumor was diagnosed as an
adrenocortical carcinoma, and immunohistochemical analysis of steroidogenic
enzymes revealed disorganized steroidogenesis in the
tumor tissue. With
adrenocortical carcinomas, heterogeneity of individual
steroid producing
enzymes within
tumor cells can lead to hypersecretion of various
steroid intermediates, even when
steroid end products are within the normal range.