Experience in the treatment of patients with ACTH-independent macronodular bilateral adrenal hyperplasia

Cover Page

Cite item

Full Text

Abstract

Aim – to determine the optimal tactics for the examination and treatment of patients with macronadular bilateral adrenal hyperplasia.

Material and methods. The study included 11 patients with macronodular bilateral adrenal hyperplasia (main group). To compare biochemical parameters, the results of 26 healthy people were studied (control group).

Results. The patients with macronodular bilateral adrenal hyperplasia were characterized by deficiency of 11β-hydroxylase, 21-hydroxylase, 11β-hydroxysteroid dehydrogenase type 2 and increased activity of 5β-reductase compared with the control group. The optimal treatment for these patients is unilateral adrenalectomy. A comparative selective blood sampling from the central veins of the adrenals is necessary to select the side of intervention in patients with bilateral macronodular adrenal hyperplasia with ACTH-independent Cushing's syndrome.

Conclusion. The first step in the treatment of patients with bilateral macronodular adrenal hyperplasia with ACTH-independent Cushing's syndrome is unilateral adrenalectomy, which reduces the risk of developing adrenal insufficiency and subsequent hormone replacement therapy.

About the authors

Aleksandr A. Lisitcyn

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: 9213244516@mail.ru
ORCID iD: 0000-0003-2045-0044
SPIN-code: 3237-4309

PhD, Associate Professor of the Department of Faculty Surgery named after I.I. Grecov

Russian Federation, Saint Petersburg

Vyacheslav P. Zemlyanoi

North-Western State Medical University named after I.I. Mechnikov

Email: 9213244516@mail.ru
ORCID iD: 0000-0003-2329-0023

PhD, Professor, Head of the Department of Faculty Surgery named after I.I. Grekov

Russian Federation, Saint Petersburg

Sergei B. Shustov

North-Western State Medical University named after I.I. Mechnikov

Email: 9213244516@mail.ru
ORCID iD: 0000-0002-9075-8274

PhD, Professor, Head of the Adrenal Pathology Center

Russian Federation, Saint Petersburg

Lyudmila I. Velikanova

North-Western State Medical University named after I.I. Mechnikov

Email: 9213244516@mail.ru
ORCID iD: 0000-0002-9352-4035

PhD, Professor, Head of the Research Laboratory of Chromatography

Russian Federation, Saint Petersburg

Evgeniya M. Nesvit

North-Western State Medical University named after I.I. Mechnikov

Email: 9213244516@mail.ru
ORCID iD: 0000-0002-2612-1922

Surgeon

Russian Federation, Saint Petersburg

References

  1. Osswald A, Deutschbein T, Berr CM, et al. Surviving ectopic Cushing's syndrome: quality of life, cardiovascular and metabolic outcomes in comparison to Cushing's disease during long-term follow-up. Eur J Endocrinol. 2018;179(2):109-116. doi: 10.1530/EJE-18-0212
  2. Zhao Y, Guo H, Zhao Y, et al. Secreting ectopic adrenal adenoma: A rare condition to be aware of. Ann Endocrinol (Paris). 2018;79(2):75-81. doi: 10.1016/j.ando.2017.03.003
  3. O'Brien KF, DeKlotz CMC, Silverman RA. Exogenous Cushing syndrome from an unexpected source of systemic steroids. Pediatr Dermatol. 2018;35(3):196-197. doi: 10.1111/pde.13456
  4. Chiodini I, Ramos-Rivera А, Marcus АО, Yau H. Adrenal Hypercortisolism: A Closer Look at Screening, Diagnosis, and Important Considerations of Different Testing Modalities. J Endocr Soc. 2019;3(5):1097-1109. doi: 10.1210/js.2018-00382
  5. Zhou J, Zhang М, Bai Х, et al. Demographic Characteristics, Etiology, and Comorbidities of Patients with Cushing’s Syndrome: A 10-Year Retrospective Study at a Large General Hospital in China. International Journal of Endocrinology. 2019:7159696. doi: 10.1155/2019/7159696
  6. Lotfollahzadeh S, Taherian М. Adrenal Cortical Nodular Hyperplasia. 2020. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan.
  7. Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the study of adrenal tumors. European Journal of Endocrinology. 2016;175(2):G1-G34. doi: 10.1530/EJE-16-0467
  8. Christopoulos S, Bourdeau I, Lacroix А. Clinical and Subclinical ACTH-Independent Macronodular Adrenal Hyperplasia and Aberrant Hormone Receptors. Horm Res. 2005;64:119-131. doi: 10.1159/000088818
  9. Lin W, Huang HB, Wen JP, et al. Approach to Cushing’s syndrome in pregnancy: two cases of Cushing’s syndrome in pregnancy and a review of the literature. Ann Transl Med. 2019;7:490. doi: 10.21037/atm.2019.07.94
  10. Zhang Q, Xiao H, Zhao L, et al. Analysis of clinical and pathological features of primary bilateral macronodular adrenocortical hyperplasia compared with unilateral cortisol-secreting adrenal adenoma. Ann Transl Med. 2020;8(18):1173. doi: 10.21037/atm-20-5963
  11. Ueland GÅ, Methlie P, Jøssang DE, et al. Adrenal venous sampling for assessment of autonomous cortisol secretion. J Clin Endocrinol Metab. 2018;103(12):4553-4560. doi: 10.1210/jc.2018-01198
  12. Acharya R, Dhir M, Bandi R, et al. Outcomes of Adrenal Venous Sampling in Patients with Bilateral Adrenal Masses and ACTH-Independent Cushing’s Syndrome. World J Surg. 2019;43:527-533. doi.org/10.1007/s00268-018-4788-2
  13. Lacroix A. ACTH-independent macronodular adrenal hyperplasia. Best Pract Res Clin Endocrinol Metab. 2009;23(2):245-259. doi: 10.1016/j.beem.2008.10.011
  14. Rushworth RL, Torpy DJ, Falhammar H. Adrenal Crisis. N Engl J Med. 2019;381(9):852-861. doi: 10.1007/s12020-016-1204-2
  15. Debillon E, Velayoudom-Cephise FL, Salenave S, et al. Unilateral Adrenalectomy as a first-line treatment of Cushing’s syndrome in patients with primary bilateral macronodular adrenal hyperplasia. J Clin Endocrinol Metab. 2015;100(12):4417-4424. doi: 10.1210/jc.2015-2662
  16. Tanno FY, Srougi V, Almeida MQ, et al. A New Insight into the Surgical Treatment of Primary Macronodular Adrenal Hyperplasia. J Endocr Soc. 2020;4(8):1-14. doi: 10.1210/jendso/bvaa083

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Figure 1. Axial (1) and coronal (2) views of the right and left adrenal glands (native phase) with bilateral macronodular hyperplasia.

Download (1MB)
3. Figure 2. Abdominal and retroperitoneal CT scan (axial view, native phase) and angiography of the right and left adrenal glands in a patient with bilateral macronodular adrenal hyperplasia with Cushing's syndrome.

Download (1MB)
4. Figure 3. Аdrenal gland with macronodular hyperplasia: 1) view of the altered adrenal gland from the side of the capsule, 2) sectional view.

Download (2MB)

Copyright (c) 2023 Lisitcyn A.A., Zemlyanoi V.P., Shustov S.B., Velikanova L.I., Nesvit E.M.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

Согласие на обработку персональных данных

 

Используя сайт https://journals.rcsi.science, я (далее – «Пользователь» или «Субъект персональных данных») даю согласие на обработку персональных данных на этом сайте (текст Согласия) и на обработку персональных данных с помощью сервиса «Яндекс.Метрика» (текст Согласия).