Adrenocortical Carcinoma with Feminization and Hypertension Associated with a Defect in 11β-Hydroxylation

Localization of Steroidogenic Enzymes in Adrenal Cortex and Its Disorders.

“…No independent markers of adrenocortical steroidogenesis have not been reported as described above but steroid metabolism in human adrenocortical carcinoma exhibits a relatively characteristic feature compared to that of both adrenocortical adenoma and the normal adrenal cortex. This feature has been summarized as low efficiency of steroidogenesis, including overproduction of minor products of adrenocortical steroidogenesis or precursor steroids [15,[34][35][36][37]. In our recent study of localization of steroidogenic enzymes in nine cases of human adrenocortical malignancy [38], a number of carcinoma cells did not express all the enzymes required for the synthesis of biologically active corticosteroids (Figs.…”

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

“…Indeed, in evidence of P450cll deficiency the tumour tissue showed a marked decrease in P450cll mRNA with normal amounts of mRNA of the other steroidogenic enzymes studied including 3[3-hydroxysteroid dehydrogenase. To our knowledge virilizing adrenal tumours mimicking congenital adrenal hyperplasia with P450c21-and P450cll deficiency have been described only in adults [12,13,15,21,26]. Such tumours, however, may occur in children and adults treated for congenital adrenal hyperplasia [7,8,17,18].…”

“…Some adult patients with adrenocortical neoplasms, however, have been described with evidence of P450c21-or P450c 11 deficiency limited to the tumour tissue [12,13,15,21,26].…”

In vitro Studies of Enzymatic Activities in Human Adrenal Tumors

“…Deficiencies of specific enzymes involved in steroidogene sis have been implicated to explain the steroidogenetic properties of the tumor [2,6,13]. In order to correlate in vitro enzyme activity with clinical data we measured sev eral enzyme activities in human adrenal tis sue obtained from patients with various pituitary/adrenal disorders.…”