285 A Virilizing Adrenocortical Tumor in a Female Infant: Steroidogenesis In Vivo and In Vitro

Abstract

Normal adrenal and adrenal tumor cells from a female infant with a virilizing adrenal tumor were grown in tissue culture for a period of 7 weeks with and without ACTH (0.1 unit/ml). The cells grew well and continued to produce steroid hormones, as measured by radioimmunoassay of individual steroids in the culture medium. Compared to normal cells, tumor cells with no ACTH added produced equivalent amounts of cortisol, 180H corticosterone, and 180H deoxycorticosterone, but lesser amounts of dehydroepiandosterone (DHA), androstenedione (A), testosterone (T) and progesterone. Normal cells exposed to ACTH showed an increase in all steroids measured whereas ACTH-exposed tumor cells showed an increase principally in DHA, consistent with a deficiency in 3β-hydroxysteroid dehydrogenase. Concentrations of DHA, A and T in the patient's serum were elevated before the adrenal tumor was removed. The relative concentration of the three androgens in media of tumor cells in vitro resembled that in patient's serum in vivo. These studies demonstrate that both normal and tumor cells of adrenal can be maintained for a long time in vitro, that they retain their ability to respond to ACTH and that they produce their characteristic steroids. The tumor cells appear to have a 3β-hydroxysteroid dehydrogenase deficiency.

Supported by the National Foundation, the Grant Foundation, and USPHS Grant RR00128, Div. Research Resources, NIH.

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