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Role of adrenal venous sampling in the differential diagnosis and treatment protocol of ACTH-independent Cushing’s syndrome with bilateral adrenal lesions

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Abstract

Purpose

The diagnosis and management of adrenocorticotropic hormone-independent Cushing’s syndrome (AICS) with bilateral adrenal lesions remain challenging. Some studies have explored the value of adrenal vein sampling (AVS) in patients with AICS; however, more investigations are needed to assess its benefits for diagnosis and treatment planning in this population.

Methods

Thirteen patients with clinical, biochemical and imaging evidence of AICS with bilateral adrenal lesions underwent AVS in our department from 2017–2022 were recruited. Only the data from nine patients for whom AVS succeeded were finally included in this study and further analyzed. Blood samples were successfully collected from both adrenal veins (AV) and inferior vena cava (IVC) in these nine patients, and the levels of plasma total cortisol (PTC) and plasma aldosterone concentrations (PAC) were measured. The ratio of the PAC of the AV to the IVC was calculated, and the PTC to PAC ratios were compared between AV. The surgical strategy was chosen according to the results of AVS. Postoperative histology and immunohistochemistry of the adrenal tissues were performed. The prognosis was evaluated based on the improvement of clinical symptoms and biochemical parameters (including PTC and ACTH measurements).

Results

Patients with AICS were clinically diagnosed based on clinical signs, results of functional tests and the presence of bilateral adrenal lesions as observed on computed tomography imaging. An AV to IVC PAC ratio greater than 2 confirmed successful AVS. The PTC to PAC ratio (high side to low side) was greater than 2 in four patients, and less than 2 in five patients. The postoperative pathological results were consistent with clinical diagnosis and AVS. During the mean follow-up of 33 months, all nine patients achieved varying degrees of clinical improvement.

Conclusion

Our study showed that AVS helped to distinguish unilateral and bilateral lesions, identify the laterality of the autonomous hypercortisolism, and improve therapeutic strategy selection in patients with AICS and bilateral adrenal lesions.

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Funding

This work was supported by the National Key Research and Development Program of China (No. 2021YFC2501600, 2021YFC2501601), the Sichuan Science and Technology Program (No. 23ZDYF2116, 23ZDYF2910), and the 1·3·5 project for disciplines of excellence–Clinical Research Incubation Project, West China Hospital, Sichuan University (No. 2021HXFH008).

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Author notes

  1. These authors contributed equally: Xingxing An, Tao Chen

Authors and Affiliations

  1. Department of Endocrinology and Metabolism, Adrenal Center, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, P. R. China

    Xingxing An, Tao Chen, Haoming Tian, Yuanmei Li, Jianwei Li, Nianwei Wu & Yan Ren

  2. Chinese Evidence-based Medicine Center, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, P. R. China

    Dan Mo

  3. Department of Urology, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, P. R. China

    Sikui Shen & Yuchun Zhu

  4. Department of Endocrinology and Metabolism, Tibet Branch of West China Hospital of Sichuan University, Chengdu, Sichuan, 610041, P. R. China

    Dan Zhang

  5. Health Management Center, General Practice Medical Center, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, P. R. China

    Tingting Zhang

  6. Institute of Clinical Pathology, West China Hospital of Sichuan University, Chengdu, 610041, China

    Li Li

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  1. Xingxing An
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Contributions

Y.R. and T.C. were mainly responsible for research design, and for elucidating the experimental findings derived from AVS investigations. X.A., D.M., T.Z. and J.L. executed the patient recruitment and longitudinal follow-up. Y.Z. was mainly conducted AVS procedures and surgical operations for all patients. S.S. took part in all AVS and surgeries. D.Z. and Y.L. were mainly responsible for data analysis. H.T. provided expert guidance in the realm of clinical diagnosis. L.L. was mainly responsible for immumohistochemical staining and subsequent pathologic diagnose. N.W. took part in AVS procedures of two patients. X.A. wrote the initial manuscript draft. X.A., T.C. and Y.R. revised the manuscript. Y.R. edited and approved the final draft.

Corresponding authors

Correspondence to Yuchun Zhu or Yan Ren.

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Conflict of interest

The authors declare no competing interests.

Ethics approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of West China Hospital.

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Informed consent was obtained from all individual participants included in the study.

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An, X., Chen, T., Mo, D. et al. Role of adrenal venous sampling in the differential diagnosis and treatment protocol of ACTH-independent Cushing’s syndrome with bilateral adrenal lesions. Endocrine 81, 562–572 (2023). https://doi.org/10.1007/s12020-023-03395-7

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  • DOI: https://doi.org/10.1007/s12020-023-03395-7

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