Production and origin of estrogen in two true hermaphrodites

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.