A case of adrenal feminization due to adenomatous hyperplasia of the adrenal cortex

Abstract

1) A case of adrenal feminizing syndrome due to adrenocortical adenomatous hyperplasia in a 27-year-old man is reported. This case is regarded as the first case caused by adenomatous hyperplasia in the world.2) The symptom was associated with gynecomastia, hypoplastic genitalia and hypertension. Enlargement of the right adrenal gland was proved by the tomography with pneumoretro-peritoneum.3) The urinary 17-KS and 17-OHCS excretion level reviewed within normal range and/or slightly lower than the normal. Androsterone was significantly decreased and dehydroepiandrosterone was increased out of fractions of urinary 17KS. Estrogen and pregnanediol were elevated. Among the fractions of urinary estrogen, estriol was increased. Urinary 17KS, 17-OHCS and estrogen excretions did dnot respond to ACTH-test, dexamethasone-test, metopirone-test or HCG-test, suggesting that secretionn of the adrenocortical hormone in this case is independent of the hypophysis.4) The right adrenalectomy was carried out and the removed specimen measured 3.0×2.5×2.0 cm and weighed 18 g. The histological diagnosis was adenomatous hyperplasia of the adrenal cortex and no sign of malignancy was detected.5) Postoperatively, the urinary 17-KS, 17-OHCS and the fractions of 17-KS were unchanged, however estrogen returned to normal level and pregnanediol showed to be lower than the pre-operative level.6) No change of clinical symptoms was noticed after the adrenalectomy, but significant improvement appeared by androgen therapy. A plastic operation for hypospadias was carried out. The hypertension in this case was difficult to control by antihypertensive agents, however the blood pressure was, controlled by the drugs within normal range after adrenalectomy.7) It is suggested that the biosynthesis of adrenal androgen in this case may pass the way from pregnenolone to androstenedione via progesterone besides the way via dehydroepiandrosterone.8) Clinical symptoms, findings of X-ray films and results of hormonal examinations of the cases of carcinoma, adenoma and our case were reviewed. Differential diagnosis from the testicular feminization and the problem of hypospadias were discussed.