副腎皮質の腺腫状過形成による女性化症の1例

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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. En largement 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 17 KS . Estrogen and pregnanediol were elevated. Among the fractions of urinary estrogen, estriol was increased. Urinary 17KS, 17-OHCS and estrogen excretions did not respond to ACTH-test, dexamethasone-test, metopirone-test or HCG-test, suggesting that secretion 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 hyperplasiaofthe 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 belower than the preoperative level. 6) No change of clinical symptoms was noticed after the adrenal ectomy, but significant improvement appeared by androgentherapy. 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.

副腎皮質の腺腫状過形成による女性化症の1例

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