Two 46,XX true hermaphrodites with
genital ambiguity since birth and
gynecomastia were studied. The production rates of
estradiol in these subjects, 77 and 71 microgram per 24 hours, and of
estrone, 82 and 136 microgram per 24 hours, were approximately twice those of normal adult men. In one of these subjects the plasma production rates of
androstenedione and
testosterone were measured and found to be 389 and 1,271 microgram per 24 hours, respectively. In this subject all
estrone production, 82 microgram per 24 hours, could be accounted for by extraglandular formation from plasma prehormones, whereas 71 microgram of
estradiol per 24 hours could not be accounted for by extraglandular formation and presumably arose from glandular secretion. The concentration of
testosterone in the testicular portion of the ovotestis of this subject was 465 ng. per gram of tissue, a value comparable to that found in testicular tissue obtained from adult men. In the other subject of this study, who had a malignant gonadal
tumor, the plasma concentration of
human chorionic gonadotropin was 3,000 ml.U. per milliliter. From the results of this study we conclude that both ovarian and testicular components of the gonads of these two individuals were endocrinologically active, and that their
gynecomastia likely developed as a consequence of gonadal
estradiol secretion.