STEROID EXCRETION AND BIOSYNTHESIS WITH SPECIAL REFERENCE TO C19-Δ16-STEROIDS IN AN INFANT WITH A VIRILIZING ADRENOCORTICAL CARCINOMA

Abstract

ABSTRACT A case of a 15-month old girl with a virilizing adrenocortical carcinoma is described. Three C19-Δ16-steroids, 3α-hydroxy-5α-androst-16-diene, 3α-hydroxy-5β-androst-16-ene and 3β-hydroxy-androsta-5,16-diene, have been obtained from the glucuronide fraction of the urine and identified by column and gas-liquid chromatographic techniques. Estimation of these compounds, not normally present in infants' urine, showed that adult female amounts were being excreted, although post-operatively very little was detected. Androsterone, aetiocholanolone and especially dehydroepiandrosterone were present in excessive amounts preoperatively; approximately one-third of the dehydroepiandrosterone was excreted as glucuronide, the rest as sulphate. The adrenal tumour metabolized [4-14C] pregnenolone and [4-14C] progesterone to 4.7% and 0.15% respectively of 3β=hydroxy-androsta-5.16-diene. The evidence for biosynthesis of this compound from progesterone supports that obtained by other workers for the production of Δ5- from Δ4-steroids. Labelled androstenedione was also obtained from both substrates and 1 1β-hydroxyandrostenedione from progesterone. Only small quantities of radioactivity were recovered in the glucuronide and sulphate fractions from the tissue incubations.