Abstract
Background
ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing’s syndrome. Bilateral adrenalectomy is considered the treatment of choice, with subsequent lifetime steroid replacement, but unilateral adrenalectomy has been recently proposed to reduce the secreting tissue. This prospective study was designed to evaluate the long-term results of unilateral adrenalectomy in AIMAH concerning the main laboratory and clinical abnormalities and the patient’s quality of life.
Methods
Seven consecutive patients with confirmed AIMAH underwent unilateral adrenalectomy of the largest gland. ACTH and cortisol levels, arterial blood pressure (BP), glycometabolic parameters, and patient’s subjective perception of health-related quality of life (by the SF-36 questionnaire) were measured preoperatively and postoperatively.
Results
No surgery-related morbidity occurred. One patient with a large contralateral adrenal enlargement required a completion adrenalectomy after 7 months because of persistent hypercortisolism. At a median follow-up of 53 (range, 27–68) months, the remaining six patients were cured, because serum and urinary free cortisol levels significantly decreased and ACTH increased, thus regaining the normal range. Both systolic and diastolic BP levels significantly reduced: 50% of patients definitively became normotensive, and the remaining patients reduced the need for antihypertensive treatment; 40% of patients suffering from preoperative diabetes were cured, whereas 40% reduced the need for hypoglycemizing drug. SF-36 evaluation of the health-related quality of life confirmed a significant amelioration.
Conclusions
Unilateral adrenalectomy of the largest gland can be an effective and safe treatment for AIMAH in case of asymmetric involvement. It may achieve long-term remission of Cushing’s syndrome and improve BP values, glycemic control, and patient’s quality of life.
Similar content being viewed by others
References
Christopoulos S, Bourdeau I, Lacroix A (2005) Clinical and subclinical ACTH-independent macronodular adrenal hyperplasia and aberrant hormone receptors. Horm Res 64:119–131
Lacroix A, N’Diaye N, Tremblay J, et al. (2001) Ectopic and abnormal hormone receptors in adrenal Cushing’s syndrome. Endocr Rev 22:75–110
Lieberman SA, Eccleshall TR, Feldman D (1994) ACTH-independent massive bilateral adrenal disease (AIMBAD): a subtype of Cushing’s syndrome with major diagnostic and therapeutic implications. Eur J Endocrinol 131:67–73
Swain JM, Grant CS, Schlinkert RT, et al. (1998) Corticotropin-independent macronodular adrenal hyperplasia: a clinicopathologic correlation. Arch Surg 133:541–545
Stratakis CA, Kirschner LS (1998) Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease). Horm Metab Res 30:456–463
Lacroix A, Tremblay J, Rousseau G, et al. (1997) Brief report: propranolol therapy for ectopic (beta)-adrenergic receptors in adrenal Cushing’s syndrome. N Engl J Med 337:1429–1434
N’Diaye N, Hamet P, Tremblay J, et al. (1999) Asynchronous development of bilateral nodular adrenal hyperplasia in gastric inhibitory polypeptide-dependent Cushing’s syndrome. J Clin Endocrinol Metab 84:2616–2622
Doppman JL, Chrousos GP, Papanicolaou DA, et al. (2000) Adrenocorticotropin-independent macronodular adrenal hyperplasia: an uncommon cause of primary adrenal hypercortisolism. Radiology 216:797–802
Lamas C, Alfaro JJ, Lucas T, et al. (2002) Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia? Long-term follow-up of four cases. Eur J Endocrinol 146:237–240
Imohl M, Koditz R, Stachon A, et al. (2002) Catecholamine-dependent hereditary Cushing’s syndrome: follow-up after unilateral adrenalectomy. Med Klin 97:747–753
Ogura M, Kusaka I, Nagasaka S, et al. (2003) Unilateral adrenalectomy improves insulin resistance and diabetes mellitus in a patient with ACTH-independent macronodular adrenal hyperplasia. Endocr J 50:715–721
Sato M, Soma M, Nakayama T, et al. (2006) A case of adrenocorticotropin-independent bilateral adrenal macronodular hyperplasia (AIMAH) with primary hyperparathyroidism. Endocr J 53:111–117
Vezzosi D, Cartier D, Regnier C, et al. (2007) Familial adrenocorticotropin-independent macronodular adrenal hyperplasia with aberrant serotonin and vasopressin adrenal receptors. Eur J Endocrinol 156:21–31
Arnaldi G, Angeli A, Atkinson AB, et al. (2003) Diagnosis and complications of Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab 88:5593–5602
Guidelines Committee (2003) 2003 European Society of Hypertension-European Society of Cardiology guidelines for the management of arterial hypertension. J Hypertens 21:1011–1053
Apolone G, Mosconi P (1998) The italian SF-36 health survey: translation, validation and norming. J Clin Epidemiol 11:1025–1036
Kirschner MA, Powell RD, Lipsett MB (1964) Cushing’s syndrome: nodular cortical hyperplasia of adrenal glands with clinical and pathological features suggesting adrenocortical tumor. J Clin Endocrinol Metab 24:947–955
Nagesser SK, van Seters AP, Kievit J, et al. (2000) Long-term results of total adrenalectomy for Cushing’s disease. World J Surg 24:108–113
Hawn MT, Cook D, Deveney C, et al. (2002) Quality of life after laparoscopic bilateral adrenalectomy for Cushing’s disease. Surgery 132:1064–1068
Morioka M, Ohashi Y, Watanabe H, et al. (1997) ACTH-independent macronodular adrenocortical hyperplasia (AIMAH): report of two cases and the analysis of steroidogenic activity in adrenal nodules. Endocr J 44:65–72
Iacobone M, Mantero F, Basso SM, et al. (2005) Results and long-term follow-up after unilateral adrenalectomy for ACTH-independent hypercortisolism in a series of fifty patients. J Endocrinol Invest 28:327–332
Ohashi A, Yamada Y, Sakaguchi K, et al. (2001) A natural history of adrenocorticotropin-independent bilateral adrenal macronodular hyperplasia (AIMAH) from preclinical to clinically overt Cushing’s syndrome. Endocr J 48:677–683
Erbil Y, Ademoglu E, Ozbey N, et al. (2006) Evaluation of the cardiovascular risk in patients with subclinical Cushing syndrome before and after surgery. World J Surg 30:1665–1671
Tauchmanova L, Rossi R, Biondi B, et al. (2002) Patients with subclinical Cushing’s syndrome due to adrenal adenoma have increased cardiovascular risk. J Clin Endocrinol Metab 87:4872–4878
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Iacobone, M., Albiger, N., Scaroni, C. et al. The Role of Unilateral Adrenalectomy in ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH). World J Surg 32, 882–889 (2008). https://doi.org/10.1007/s00268-007-9408-5
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00268-007-9408-5