Adrenocorticotropin-Independnt Bilateral Adrenal Macronodular Hyperplasia (AIMAH): A Case Report

非促腎上腺皮質激素依賴型大結節性腎上腺皮質過度增生：病例報告

謝旻晃(Min-Huang Hsieh) ； 張慶忠(Ching-Chung Chang) ； 林明杰(Min-Chieh Lin) ； 張天鈞(Tien-Chun Chang)

《內科學誌》 17卷6期 (2006/12) Pp. 291-297

https://doi.org/10.6314/JIMT.2006.17(6).04

摘要

非促腎上腺皮質激素依賴型大結節性腎上腺皮質過度增生是造成庫欣氏症候群一個很罕見的原因。它的特色是臨床上有庫欣氏症候群的表現，生化檢查會發現高皮質醇血症和低促腎上腺皮質激素，影像學上常可見兩側腎上腺結節性腫大。診斷的標凖還是要靠病理組織檢查。我們在此報告一位患有高血壓的53歲女性病人被診斷為非促腎上腺皮質激素依賴型大結節性腎上腺皮質過度增生。接受經內試鏡兩側腎上腺切除和類固醇補充治療後，病人自覺整體狀況良好，而且她的血壓變得比較好控制，體重也逐漸下降。目前她還在門診持續追蹤治療中。

並列摘要

Background: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome (CS), which is characterized by massive bilateral nodular enlargement of adrenal glands and hypercortisolism in the presence of suppressed ACTH levels. Case: A 53 year-old woman with a 5-year history of hypertension was referred to our hospital for further evaluation of bilateral adrenal tumors with macronodules, which were detected by computed tomography. She presented with a weight gain of 3 kg over the previous 6 months and pitting edema. Endocrinological data of the patient showed ACTH-independent hypercortisolemia. CS due to AIMAH was suspected. She underwent simultaneous bilateral laparoscopic adrenalectomy. The histopathological analysis confirmed the diagnosis. She was on cortisone acetate (37.5mg per day) replacement therapy postoperatively. No hyperpigmentation was present and she was being well. Her weight declined gradually and her blood pressure was controlled better with less doses of antihypertensive agents during follow-up. Conclusion: AIMAH is an unusual type of Cushing's syndrome. Bilateral adrenalectomy with subsequent steroid replacement is the treatment of choice in patients with AIMAH and CS.

並列關鍵字

ACTH-independent macronodular adrenal hyperplasia AIMAH ； Cushing's syndrome CS ； Laparoscopic adrenalectomy