Study of Δ5, 3β-Hydroxysteroid Dehydrogenase in Normal, Hyperplastic and Neoplastic Adrenal Cortical Tissue

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Abstract
Normal, hyperplastic and neoplastic adrenal cortical tissues have been studied histochemically and biochemically for activity of [DELTA]5,3[beta]-hydroxysteroid dehydrogenase (3[beta]-HSD) and biochemically for activity of 21- and 11-steroid hydroxylases (21-OHase, 11-OHase). A selective deficiency of 3[beta] -HSD activity has been demonstrated by both methods in the tissues of the patients with adrenal disease associated with the excretion of abnormally high levels of [DELTA]5,3[beta]-hydroxysteroids. These include the testes and hyperplastic adrenals of a hypospadic male infant with the rare type of "salt-losing" adrenogenital syndrome, several virilizing adrenal adenomas and a virilizing adrenal carcinoma. In a patient with the usual form of adrenogenital syndrome, staining for activity of 3[beta]-HSD was demonstrated throughout his hyperplastic adrenals. This patient''s adrenal showed a selective deficiency of 21-OHase activity biochemically. The virilizing carcinoma of the patient without excess 45,3[beta]-hydroxysteroid excretion exhibited activity of all 3 enzymes biochemically, and focal staining for activity of 3[beta]-HSD of slight to moderate intensity. The recurrence of this carcinoma a half year later showed markedly increased activities of all 3 enzymes biochemically and a high intensity of staining of 3[beta] -HSD throughout the section. Staining for activity of 3[beta] -HSD occurs in the neocortex of a liveborn 6-month-premature infant and of normal neonates and is absent in the fetal zone which comprises the bulk of the adrenal cortex at these ages. In older children, staining for activity of 3[beta]-HSD is most intense in the outer half of the cortex and occurs occasionally in scattered areas of the zona reticularis.