Skip to main content
Top
Published in: BMC Endocrine Disorders 1/2018

Open Access 01-12-2018 | Case report

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

Authors: Jia Wei, Sheyu Li, Qilin Liu, Yuchun Zhu, Nianwei Wu, Ying Tang, Qianrui Li, Kaiyun Ren, Qianying Zhang, Yerong Yu, Zhenmei An, Jing Chen, Jianwei Li

Published in: BMC Endocrine Disorders | Issue 1/2018

Login to get access

Abstract

Background

Adrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome (CS) with bilateral cortisol-secreting adenomas has been rarely reported in the literatures. Precise recognition and management of this disorder constitute a challenge to clinicians due to the difficulty of exact location of the functional lesions.

Case presentation

We herein report a new case of a Chinese female patient with a complaint of exertional dyspnea for over 10 years. ACTH-independent CS was diagnosed based on undetectable ACTH and unsuppressed cortisol levels by dexamethasone. Computed tomography (CT) scan indicated bilateral adrenal masses, and adrenal venous sampling (AVS) adjusted by plasma aldosterone revealed hypersecretion of cortisol from both adrenal glands. Bilateral cortisol-secreting adrenal adenomas were suspected and confirmed by the postoperative pathology in subsequent two-step bilateral laparoscopic adrenalectomy. The symptoms and signs of CS relieved after surgery with continuous glucocorticoid replacement.

Conclusions

AVS adjusted by plasma aldosterone could be a useful technique in diagnosing ACTH-independent CS with bilateral adrenal adenomas prior to surgery. And the aldosterone ratio could be used to confirm the success of adrenal vein cannulation in this situation.
Literature
1.
go back to reference Newell-Price J, Bertagna X, Grossman AB, Nieman LK. Cushing's syndrome. Lancet. 2006;367(9522):1605–17.CrossRefPubMed Newell-Price J, Bertagna X, Grossman AB, Nieman LK. Cushing's syndrome. Lancet. 2006;367(9522):1605–17.CrossRefPubMed
2.
go back to reference Graversen D, Vestergaard P, Stochholm K, Gravholt CH, Jorgensen JO. Mortality in Cushing's syndrome: a systematic review and meta-analysis. Eur J Intern Med. 2012;23(3):278–82.CrossRefPubMed Graversen D, Vestergaard P, Stochholm K, Gravholt CH, Jorgensen JO. Mortality in Cushing's syndrome: a systematic review and meta-analysis. Eur J Intern Med. 2012;23(3):278–82.CrossRefPubMed
3.
go back to reference Lacroix A, Bourdeau I. Bilateral adrenal Cushing's syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease. Endocrinol Metab Clin N Am. 2005;34(2):441–58.CrossRef Lacroix A, Bourdeau I. Bilateral adrenal Cushing's syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease. Endocrinol Metab Clin N Am. 2005;34(2):441–58.CrossRef
4.
go back to reference Doppman JL. The dilemma of bilateral adrenocortical nodularity in Conn's and Cushing's syndromes. Radiol Clin N Am. 1993;31(5):1039–50.PubMed Doppman JL. The dilemma of bilateral adrenocortical nodularity in Conn's and Cushing's syndromes. Radiol Clin N Am. 1993;31(5):1039–50.PubMed
5.
go back to reference Maghrabi A, Yaqub A, Denning KL, Benhamed N, Faiz S, Saleem T. Challenges in the diagnostic work-up and management of patients with subclinical Cushing's syndrome and bilateral adrenal masses. Endocr Pract. 2013;19(3):515–21.CrossRefPubMed Maghrabi A, Yaqub A, Denning KL, Benhamed N, Faiz S, Saleem T. Challenges in the diagnostic work-up and management of patients with subclinical Cushing's syndrome and bilateral adrenal masses. Endocr Pract. 2013;19(3):515–21.CrossRefPubMed
6.
go back to reference Tung SC, Wang PW, Huang TL, Lee WC, Chen WJ. Bilateral adrenocortical adenomas causing ACTH-independent Cushing's syndrome at different periods: a case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy. J Endocrinol Investig. 2004;27(4):375–9.CrossRef Tung SC, Wang PW, Huang TL, Lee WC, Chen WJ. Bilateral adrenocortical adenomas causing ACTH-independent Cushing's syndrome at different periods: a case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy. J Endocrinol Investig. 2004;27(4):375–9.CrossRef
7.
go back to reference Imai T, Funahashi H, Tanaka Y, Tobinaga J, Wada M, Morita-Matsuyama T, et al. Adrenalectomy for treatment of Cushing syndrome: results in 122 patients and long-term follow-up studies. World J Surg. 1996;20(7):781–6. discussion 786-7CrossRefPubMed Imai T, Funahashi H, Tanaka Y, Tobinaga J, Wada M, Morita-Matsuyama T, et al. Adrenalectomy for treatment of Cushing syndrome: results in 122 patients and long-term follow-up studies. World J Surg. 1996;20(7):781–6. discussion 786-7CrossRefPubMed
8.
go back to reference Young WF Jr, du Plessis H, Thompson GB, Grant CS, Farley DR, Richards ML, et al. The clinical conundrum of corticotropin-independent autonomous cortisol secretion in patients with bilateral adrenal masses. World J Surg. 2008;32(5):856–62.CrossRefPubMed Young WF Jr, du Plessis H, Thompson GB, Grant CS, Farley DR, Richards ML, et al. The clinical conundrum of corticotropin-independent autonomous cortisol secretion in patients with bilateral adrenal masses. World J Surg. 2008;32(5):856–62.CrossRefPubMed
9.
go back to reference Domino JP, Chionh SB, Lomanto D, Katara AN, Rauff A, Cheah WK. Laparoscopic partial adrenalectomy for bilateral cortisol-secreting adenomas. Asian J Surg. 2007;30(2):154–7.CrossRefPubMed Domino JP, Chionh SB, Lomanto D, Katara AN, Rauff A, Cheah WK. Laparoscopic partial adrenalectomy for bilateral cortisol-secreting adenomas. Asian J Surg. 2007;30(2):154–7.CrossRefPubMed
10.
go back to reference Oki K, Yamane K, Sakashita Y, Kamei N, Watanabe H, Toyota N, et al. Primary aldosteronism and hypercortisolism due to bilateral functioning adrenocortical adenomas. Clin Exp Nephrol. 2008;12(5):382–7.CrossRefPubMed Oki K, Yamane K, Sakashita Y, Kamei N, Watanabe H, Toyota N, et al. Primary aldosteronism and hypercortisolism due to bilateral functioning adrenocortical adenomas. Clin Exp Nephrol. 2008;12(5):382–7.CrossRefPubMed
12.
go back to reference Mimou N, Sakato S, Nakabayashi H, Saito Z, Takeda R, Matsubara F. Cushing's syndrome associated with bilateral adrenal adenomas. Acta Endocrinol. 1985;108(2):245–54.PubMed Mimou N, Sakato S, Nakabayashi H, Saito Z, Takeda R, Matsubara F. Cushing's syndrome associated with bilateral adrenal adenomas. Acta Endocrinol. 1985;108(2):245–54.PubMed
13.
go back to reference Dinneen SF, Carney JA, Carpenter PC, Grant CS, Young WF Jr. Acth-independent Cushing's syndrome: bilateral cortisol-producing adrenal adenomas. Endocr Pract. 1995;1(2):77–81.CrossRefPubMed Dinneen SF, Carney JA, Carpenter PC, Grant CS, Young WF Jr. Acth-independent Cushing's syndrome: bilateral cortisol-producing adrenal adenomas. Endocr Pract. 1995;1(2):77–81.CrossRefPubMed
14.
go back to reference Makino S, Chikazawa H, Yorimitsu A, Suehiro T, Hashimoto K, Ohashi Y, et al. A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas. Endocr J. 1997;44(4):533–40.CrossRefPubMed Makino S, Chikazawa H, Yorimitsu A, Suehiro T, Hashimoto K, Ohashi Y, et al. A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas. Endocr J. 1997;44(4):533–40.CrossRefPubMed
15.
go back to reference Tamura H, Sugihara H, Minami S, Emoto N, Shibasaki T, Shuto Y, et al. Cushing's syndrome due to bilateral adrenocortical adenomas with different pathological features. Intern Med. 1997;36(11):804–9.CrossRefPubMed Tamura H, Sugihara H, Minami S, Emoto N, Shibasaki T, Shuto Y, et al. Cushing's syndrome due to bilateral adrenocortical adenomas with different pathological features. Intern Med. 1997;36(11):804–9.CrossRefPubMed
16.
go back to reference Desai N, Kapoor A, Singh BK, Liu J. Bilateral adrenal adenomas and persistent leukocytosis: a unique case of Cushing's syndrome. Am J Med. 2006;119(3):e3–5.CrossRefPubMed Desai N, Kapoor A, Singh BK, Liu J. Bilateral adrenal adenomas and persistent leukocytosis: a unique case of Cushing's syndrome. Am J Med. 2006;119(3):e3–5.CrossRefPubMed
17.
go back to reference Martins RG, Agrawal R, Berney DM, Reznek R, Matson M, Grossman AB, et al. Differential diagnosis of adrenocorticotropic hormone-independent Cushing syndrome: role of adrenal venous sampling. Endocr Pract. 2012;18(6):e153–7.CrossRefPubMed Martins RG, Agrawal R, Berney DM, Reznek R, Matson M, Grossman AB, et al. Differential diagnosis of adrenocorticotropic hormone-independent Cushing syndrome: role of adrenal venous sampling. Endocr Pract. 2012;18(6):e153–7.CrossRefPubMed
18.
go back to reference Ku EJ, Hong AR, Kim YA, Bae JH, Chang MS, Kim SW. Adrenocorticotropic hormone-independent Cushing syndrome with bilateral cortisol-secreting adenomas. Endocrinol Metab (Seoul). 2013;28(2):133–7.CrossRef Ku EJ, Hong AR, Kim YA, Bae JH, Chang MS, Kim SW. Adrenocorticotropic hormone-independent Cushing syndrome with bilateral cortisol-secreting adenomas. Endocrinol Metab (Seoul). 2013;28(2):133–7.CrossRef
19.
go back to reference Monno S, Tachikawa T, Maekawa T, Sasano H. A case of Cushing's syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion. Endocr J. 2015;62(3):283–8.CrossRefPubMed Monno S, Tachikawa T, Maekawa T, Sasano H. A case of Cushing's syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion. Endocr J. 2015;62(3):283–8.CrossRefPubMed
20.
go back to reference Yasuda A, Seki T, Ito K, Takagi A, Watanabe D, Nakamura N, et al. A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas. Tokai J Exp Clin Med. 2014;39(4):158–65.PubMed Yasuda A, Seki T, Ito K, Takagi A, Watanabe D, Nakamura N, et al. A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas. Tokai J Exp Clin Med. 2014;39(4):158–65.PubMed
21.
go back to reference Inoue T, Ishiguro K, Suda T, Ito N, Suzuki Y, Taniguchi Y, et al. Laparoscopic bilateral partial adrenalectomy for adrenocortical adenomas causing Cushing's syndrome: report of a case. Surg Today. 2006;36(1):94–7.CrossRefPubMed Inoue T, Ishiguro K, Suda T, Ito N, Suzuki Y, Taniguchi Y, et al. Laparoscopic bilateral partial adrenalectomy for adrenocortical adenomas causing Cushing's syndrome: report of a case. Surg Today. 2006;36(1):94–7.CrossRefPubMed
22.
go back to reference Nomura K, Saito H, Aiba M, Iihara M, Obara T, Takano K. Cushing's syndrome due to bilateral adrenocortical adenomas with unique histological features. Endocr J. 2003;50(2):155–62.CrossRefPubMed Nomura K, Saito H, Aiba M, Iihara M, Obara T, Takano K. Cushing's syndrome due to bilateral adrenocortical adenomas with unique histological features. Endocr J. 2003;50(2):155–62.CrossRefPubMed
23.
go back to reference Li Z, Zhu Y, Kong C, Yin L, Gao Z, Zhao W, et al. Corticotropin-independent Cushing's syndrome in patients with bilateral adrenal masses. Urology. 2011;77(2):417–21.CrossRefPubMed Li Z, Zhu Y, Kong C, Yin L, Gao Z, Zhao W, et al. Corticotropin-independent Cushing's syndrome in patients with bilateral adrenal masses. Urology. 2011;77(2):417–21.CrossRefPubMed
24.
go back to reference Aiba M, Kawakami M, Ito Y, Fujimoto Y, Suda T, Demura H. Bilateral adrenocortical adenomas causing Cushing's syndrome. Report of two cases with enzyme histochemical and ultrastructural studies and a review of the literature. Arch Pathol Lab Med. 1992;116(2):146–50.PubMed Aiba M, Kawakami M, Ito Y, Fujimoto Y, Suda T, Demura H. Bilateral adrenocortical adenomas causing Cushing's syndrome. Report of two cases with enzyme histochemical and ultrastructural studies and a review of the literature. Arch Pathol Lab Med. 1992;116(2):146–50.PubMed
25.
go back to reference Horvath A, Stratakis C. Primary pigmented nodular adrenocortical disease and Cushing's syndrome. Arq Bras Endocrinol Metabol. 2007;51(8):1238–44.CrossRefPubMed Horvath A, Stratakis C. Primary pigmented nodular adrenocortical disease and Cushing's syndrome. Arq Bras Endocrinol Metabol. 2007;51(8):1238–44.CrossRefPubMed
26.
go back to reference Albiger NM, Ceccato F, Zilio M, Barbot M, Occhi G, Rizzati S, et al. An analysis of different therapeutic options in patients with Cushing's syndrome due to bilateral macronodular adrenal hyperplasia: a single-centre experience. Clin Endocrinol. 2015;82(6):808–15.CrossRef Albiger NM, Ceccato F, Zilio M, Barbot M, Occhi G, Rizzati S, et al. An analysis of different therapeutic options in patients with Cushing's syndrome due to bilateral macronodular adrenal hyperplasia: a single-centre experience. Clin Endocrinol. 2015;82(6):808–15.CrossRef
27.
go back to reference Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, et al. Treatment of Cushing's syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2015;100(8):2807–31.CrossRefPubMedPubMedCentral Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, et al. Treatment of Cushing's syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2015;100(8):2807–31.CrossRefPubMedPubMedCentral
28.
go back to reference Lumachi F, Zucchetta P, Marzola MC, Bui F, Casarrubea G, Angelini F, et al. Usefulness of CT scan, MRI and radiocholesterol scintigraphy for adrenal imaging in Cushing's syndrome. Nucl Med Commun. 2002;23(5):469–73.CrossRefPubMed Lumachi F, Zucchetta P, Marzola MC, Bui F, Casarrubea G, Angelini F, et al. Usefulness of CT scan, MRI and radiocholesterol scintigraphy for adrenal imaging in Cushing's syndrome. Nucl Med Commun. 2002;23(5):469–73.CrossRefPubMed
29.
go back to reference Builes-Montano CE, Villa-Franco CA, Roman-Gonzalez A, Velez-Hoyos A, Echeverri-Isaza S. Adrenal venous sampling in a patient with adrenal Cushing syndrome. Colomb Med (Cali). 2015;46(2):84–7. Builes-Montano CE, Villa-Franco CA, Roman-Gonzalez A, Velez-Hoyos A, Echeverri-Isaza S. Adrenal venous sampling in a patient with adrenal Cushing syndrome. Colomb Med (Cali). 2015;46(2):84–7.
30.
go back to reference Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2016;101(5):1889–916.CrossRefPubMed Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2016;101(5):1889–916.CrossRefPubMed
31.
go back to reference Sarkar SD, Beierwaltes H, Ice RD, Basmadjian GP, Hetzel KR, Kennedy WP, et al. A new and superior adrenal scanning agent, NP-59. J Nucl Med. 1975;16(11):1038–42.PubMed Sarkar SD, Beierwaltes H, Ice RD, Basmadjian GP, Hetzel KR, Kennedy WP, et al. A new and superior adrenal scanning agent, NP-59. J Nucl Med. 1975;16(11):1038–42.PubMed
32.
go back to reference Nishikawa T, Omura M, Satoh F, Shibata H, Takahashi K, Tamura N, et al. Guidelines for the diagnosis and treatment of primary aldosteronism--the Japan endocrine society 2009. Endocr J. 2011;58(9):711–21.CrossRefPubMed Nishikawa T, Omura M, Satoh F, Shibata H, Takahashi K, Tamura N, et al. Guidelines for the diagnosis and treatment of primary aldosteronism--the Japan endocrine society 2009. Endocr J. 2011;58(9):711–21.CrossRefPubMed
33.
go back to reference Funder JW, Carey RM, Fardella C, Gomez-Sanchez CE, Mantero F, Stowasser M, et al. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008;93(9):3266–81.CrossRefPubMed Funder JW, Carey RM, Fardella C, Gomez-Sanchez CE, Mantero F, Stowasser M, et al. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008;93(9):3266–81.CrossRefPubMed
34.
go back to reference Guo YW, Hwu CM, Won JG, Chu CH, Lin LY. A case of adrenal Cushing's syndrome with bilateral adrenal masses. Endocrinol Diabetes Metab Case Rep. 2016;2016:15–0118. Guo YW, Hwu CM, Won JG, Chu CH, Lin LY. A case of adrenal Cushing's syndrome with bilateral adrenal masses. Endocrinol Diabetes Metab Case Rep. 2016;2016:15–0118.
35.
go back to reference Young WF, Stanson AW, Thompson GB, Grant CS, Farley DR, van Heerden JA. Role for adrenal venous sampling in primary aldosteronism. Surgery. 2004;136(6):1227–35.CrossRefPubMed Young WF, Stanson AW, Thompson GB, Grant CS, Farley DR, van Heerden JA. Role for adrenal venous sampling in primary aldosteronism. Surgery. 2004;136(6):1227–35.CrossRefPubMed
36.
go back to reference Rossi GP, Barisa M, Allolio B, Auchus RJ, Amar L, Cohen D, et al. The Adrenal Vein Sampling International Study (AVIS) for identifying the major subtypes of primary aldosteronism. J Clin Endocrinol Metab. 2012;97(5):1606–14.CrossRefPubMed Rossi GP, Barisa M, Allolio B, Auchus RJ, Amar L, Cohen D, et al. The Adrenal Vein Sampling International Study (AVIS) for identifying the major subtypes of primary aldosteronism. J Clin Endocrinol Metab. 2012;97(5):1606–14.CrossRefPubMed
37.
go back to reference Daunt N. Adrenal vein sampling: how to make it quick, easy, and successful. Radiographics. 2005;25(Suppl 1):S143–58.CrossRefPubMed Daunt N. Adrenal vein sampling: how to make it quick, easy, and successful. Radiographics. 2005;25(Suppl 1):S143–58.CrossRefPubMed
38.
go back to reference Iihara M, Suzuki R, Kawamata A, Omi Y, Kodama H, Igari Y, et al. Adrenal-preserving laparoscopic surgery in selected patients with bilateral adrenal tumors. Surgery. 2003;134(6):1066–72. discussion 1072-73CrossRefPubMed Iihara M, Suzuki R, Kawamata A, Omi Y, Kodama H, Igari Y, et al. Adrenal-preserving laparoscopic surgery in selected patients with bilateral adrenal tumors. Surgery. 2003;134(6):1066–72. discussion 1072-73CrossRefPubMed
39.
go back to reference Li ZH, Yu XY, Zhu YY, Zhang YX, Kong CZ. Steroid replacement therapy following adrenalectomy in patients with Cushing syndrome. J China Med Uni. 2010;39(06):476–7. Li ZH, Yu XY, Zhu YY, Zhang YX, Kong CZ. Steroid replacement therapy following adrenalectomy in patients with Cushing syndrome. J China Med Uni. 2010;39(06):476–7.
40.
go back to reference Ritzel K, Beuschlein F, Mickisch A, Osswald A, Schneider HJ, Schopohl J, et al. Clinical review: outcome of bilateral adrenalectomy in Cushing's syndrome: a systematic review. J Clin Endocrinol Metab. 2013;98(10):3939–48.CrossRefPubMed Ritzel K, Beuschlein F, Mickisch A, Osswald A, Schneider HJ, Schopohl J, et al. Clinical review: outcome of bilateral adrenalectomy in Cushing's syndrome: a systematic review. J Clin Endocrinol Metab. 2013;98(10):3939–48.CrossRefPubMed
Metadata
Title
ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures
Authors
Jia Wei
Sheyu Li
Qilin Liu
Yuchun Zhu
Nianwei Wu
Ying Tang
Qianrui Li
Kaiyun Ren
Qianying Zhang
Yerong Yu
Zhenmei An
Jing Chen
Jianwei Li
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Endocrine Disorders / Issue 1/2018
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-018-0250-6

Other articles of this Issue 1/2018

BMC Endocrine Disorders 1/2018 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.