Skip to main content
Log in

The Role of Unilateral Adrenalectomy in ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)

  • Published:
World Journal of Surgery Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Christopoulos S, Bourdeau I, Lacroix A (2005) Clinical and subclinical ACTH-independent macronodular adrenal hyperplasia and aberrant hormone receptors. Horm Res 64:119–131

    Article  PubMed  CAS  Google Scholar 

  2. 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

    Article  PubMed  CAS  Google Scholar 

  3. 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

    Article  PubMed  CAS  Google Scholar 

  4. Swain JM, Grant CS, Schlinkert RT, et al. (1998) Corticotropin-independent macronodular adrenal hyperplasia: a clinicopathologic correlation. Arch Surg 133:541–545

    Article  PubMed  CAS  Google Scholar 

  5. Stratakis CA, Kirschner LS (1998) Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease). Horm Metab Res 30:456–463

    PubMed  CAS  Google Scholar 

  6. 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

    Article  PubMed  CAS  Google Scholar 

  7. 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

    Article  PubMed  CAS  Google Scholar 

  8. 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

    PubMed  CAS  Google Scholar 

  9. 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

    Article  PubMed  CAS  Google Scholar 

  10. 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

    Article  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. 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

    Article  PubMed  Google Scholar 

  13. 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

    Article  PubMed  CAS  Google Scholar 

  14. 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

    Article  PubMed  CAS  Google Scholar 

  15. Guidelines Committee (2003) 2003 European Society of Hypertension-European Society of Cardiology guidelines for the management of arterial hypertension. J Hypertens 21:1011–1053

    Article  Google Scholar 

  16. Apolone G, Mosconi P (1998) The italian SF-36 health survey: translation, validation and norming. J Clin Epidemiol 11:1025–1036

    Article  Google Scholar 

  17. 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

    Article  PubMed  CAS  Google Scholar 

  18. 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

    Article  PubMed  CAS  Google Scholar 

  19. Hawn MT, Cook D, Deveney C, et al. (2002) Quality of life after laparoscopic bilateral adrenalectomy for Cushing’s disease. Surgery 132:1064–1068

    Article  PubMed  Google Scholar 

  20. 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

    Article  PubMed  CAS  Google Scholar 

  21. 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

    PubMed  CAS  Google Scholar 

  22. 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

    Article  PubMed  CAS  Google Scholar 

  23. 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

    Article  PubMed  Google Scholar 

  24. 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

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Maurizio Iacobone.

Rights and permissions

Reprints 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

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-007-9408-5

Keywords

Navigation