Cushing Syndrome Caused by ACTH-independent Macronodular Adrenal Hyperplasia
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Adenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing’s syndrome. It is characterized by multinodular enlargement of the adrenal glands and hypercortisolism. Although bilateral adrenalectomy is the standard therapy, unilateral adrenalectomy is an effective alternative. Here we present a case of a 71-year-old female referred to the Endocrinology Department for further evaluation of bilateral adrenal macronodular hyperplasia. Based on dynamic hormone tests and imaging studies, she was diagnosed with AIMAH. Due to persistent hypercortisolism, she underwent completion contralateral surgery after unilateral adrenalectomy. This case demonstrates that unilateral adrenalectomy should be considered in a patient presenting with obvious symptoms of hypercotisolism and relatively asymmetric adrenal enlargement.
Abstract
Adenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing’s syndrome. It is characterized by multinodular enlargement of the adrenal glands and hypercortisolism. Although bilateral adrenalectomy is the standard therapy, unilateral adrenalectomy is an effective alternative. Here we present a case of a 71-year-old female referred to the Endocrinology Department for further evaluation of bilateral adrenal macronodular hyperplasia. Based on dynamic hormone tests and imaging studies, she was diagnosed with AIMAH. Due to persistent hypercortisolism, she underwent completion contralateral surgery after unilateral adrenalectomy. This case demonstrates that unilateral adrenalectomy should be considered in a patient presenting with obvious symptoms of hypercotisolism and relatively asymmetric adrenal enlargement.
참고문헌 (10)
- Newell-Price J, "Cushing's syndrome", Lancet, 367, pp.1605~1617 (2006)
- Stratakis CA, "Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease) leading to Cushing syndrome", Horm Metab Res, 30, pp.456~463 (1998)
- Iacobone M, "The role of unilateral adrenalectomy in ACTH-independent macronodular adrenal hyperplasia (AIMAH)", World J Surg, 32, pp.882~889 (2008)
- Fragoso MC, "Cushing's syndrome secondary to adrenocorticotropin-independent macronodular adrenocortical hyperplasia due to activating mutations of GNAS1 gene", J Clin Endocrinol Metab, 88, pp.2147~2151 (2003)
- Lacroix A, "Ectopic and abnormal hormone receptors in adrenal Cushing's syndrome", Endocr Rev, 22, pp.75~110 (2001)
- Louiset E, "Intraadrenal corticotropin in bilateral macronodular adrenal hyperplasia", N Engl J Med, 369, pp.2115~2125 (2013)
- Ito T, "Successful treatment for adrenocorticotropic hormone-independent macronodular adrenal hyperplasia with laparoscopic adrenalectomy: a case series", J Med Case Rep, 6, pp.312~ (2012)
- 이시훈, "일측성 부신절제로 완치된 양측성 거대결절성？부신피질증식증에 의한 쿠싱증후군 1예", Endocrinology and Metabolism, 17(4), pp.596~602 (2002), 대한내분비학회
- Lee S, "Role of unilateral aderenalectomy in ACTH-independent macronodular adrenal hyperplasia", World J Surg, 33, pp.157~158 (2009)
- Livia M. Mermejo, "ACTH-Independent Macronodular Adrenal Hyperplasia", Endocrinology and Metabolism, 26(1), pp.1~11 (2011), 대한내분비학회