A VIRILIZING ADRENAL TUMOR WITH BORDERLINE ELEVATION OF URINARY 17-KETOSTEROIDS

Abstract

In vivo and in vitro studies on a 6-year-old boy with a virilizing adrenal adenoma are reported. Of particular interest was the modest elevation of urinary 17-ketosteroids. The awareness of this observation is emphasized. The main biochemical findings were: elevated dehydroepiandrosterone sulfate (DHA-S), normal testosterone and androstenedione levels in plasma, and increased urinary excretion of dehydroepiandrosterone (DHA), 16-hydroxy-DHA, androstenediol and androstenetriol, which were isolated from the sulfate fraction. In vitro incubation of tumor tissue demonstrated its capacity to sulfurylate pregnenolone. It is postulated that DHA-S is the principal precursor of the urinary metabolites isolated, and that its metabolism in this case (possibly a function of age) led to a relatively small contribution of androsterone and etiocholanolone, in contrast to other steroids measured, particularly 16-hydroxy-DHA.