Cushing's syndromeを呈した同時性両側性副腎皮質腺腫の1例

Abstract

肥満, 腰痛を呈した43歳女で, 血漿コルチゾール値は正常で, ACTH値は低下していた.両側副腎静脈血中コルチゾール値は著明に上昇していた.CTで両側副腎腫大を認めた.131I-adosterolシンチグラム上, 右側にのみ強い取り込みを認めた.右副腎全摘によりクッシング症候群は寛解し, 病理診断は直径3.5cmの副腎皮質腺腫であった.9年でクッシング症候群が再発し, 3cmの副腎皮質腺腫を含む左副腎亜全摘を行った.経口ステロイド補充で術後回復は順調であった

A 43-year-old woman presented with obesity and lumbago. Endocrinological examinations revealed normal plasma cortisol levels and a suppressed serum adrenocorticotropic hormone (ACTH) level. On venous sampling, markedly elevated plasma cortisol levels were observed for bilateral adrenal veins (243 and 62.3 micrograms/dl on the right and left sides, respectively). Although the computed tomogram revealed bilaterally enlarged adrenal glands, 131I-adosterol scintigram showed a strong uptake only on the right side. Right adrenalectomy successfully relieved Cushing's syndrome. Pathological diagnosis was adrenocortical adenoma, 3.5 cm in diameter. Cushing's syndrome recurred in 9 years. At that time, she underwent left subtotal adrenalectomy including a 3-cm adrenocortical adenoma. Postoperative convalescence has been uneventful with oral steroid supplementation. All 14 previously reported cases of bilateral adrenocortical adenoma (BAA) causing Cushing's syndrome as well as the present case were concurrent and dominant in females of reproductive age. This suggests that some cofactors other than ACTH, such as estrogen, contribute to the pathogenesis of BAA.