Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 26, 2019; 7(8): 961-971
Published online Apr 26, 2019. doi: 10.12998/wjcc.v7.i8.961
Bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing’s syndrome: A case report and review of the literature
Yu-Lin Gu, Wei-Jun Gu, Jing-Tao Dou, Zhao-Hui Lv, Jie Li, Sai-Chun Zhang, Guo-Qing Yang, Qing-Hua Guo, Jian-Ming Ba, Li Zang, Nan Jin, Jin Du, Yu Pei, Yi-Ming Mu
Yu-Lin Gu, Wei-Jun Gu, Jing-Tao Dou, Zhao-Hui Lv, Sai-Chun Zhang, Guo-Qing Yang, Qing-Hua Guo, Jian-Ming Ba, Li Zang, Nan Jin, Jin Du, Yu Pei, Yi-Ming Mu, Department of Endocrinology, Chinese People’s Liberation Army General Hospital, Beijing 100853, China
Jie Li, Department of Pathology, Chinese People’s Liberation Army General Hospital, Beijing 100853, China
Author contributions: Gu WJ treated the patient, collected the data, and reviewed the manuscript; Gu YL analyzed the data and drafted the manuscript; Zhang SC was involved in nursing care of the patient and made substantial contributions to case report conception and design, and data acquisition, analysis, and interpretation; LJ performed the histological examination; Lv ZH, Yang GQ, Guo QH, Ba JM, Zang L, Jin N, Du J, and Pei Y made substantial contributions to case report conception and design and data acquisition, analysis, and interpretation, and revised the manuscript critically for important intellectual content; Dou JT and Mu YM were the superior advisors, and they gave final approval of the version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors read and approved the final manuscript.
Informed consent statement: All study participants, or their legal guardian, provided written informed consent prior to study enrollment. And written informed consent was obtained from the patient for publication of this report and any accompanying images. The study was reviewed and approved by the Chinese People’s Liberation Army General Hospital Institutional Review Board.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Wei-Jun Gu, MD, PhD, Assistant Professor, Department of Endocrinology, Chinese People’s Liberation Army General Hospital, Beijing 100853, China. guweijun301@163.com
Telephone: +86-010-55499301 Fax: +86-010-68168631
Received: December 2, 2018
Peer-review started: December 3, 2018
First decision: January 26, 2018
Revised: January 31, 2018
Accepted: February 26, 2019
Article in press: February 26, 2019
Published online: April 26, 2019
Abstract
BACKGROUND

Adrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome (CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease, ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas (BAAs) or carcinomas. BAAs causing ACTH-independent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling (AVS) is a good way to diagnose ACTH-independent CS.

CASE SUMMARY

A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and C-peptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin (DDVAP) stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs. Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands (right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first, followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now, all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.

CONCLUSION

BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.

Keywords: Bilateral adrenocortical adenomas, Adrenocorticotropic hormone-independent Cushing’s syndrome, Adrenal venous sampling, Case report

Core tip: Bilateral adrenocortical adenomas (BAAs) causing adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS) are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. We report a rare case of BAAs causing ACTH-independent CS, which was diagnosed by adrenal vein sampling (AVS) and computed tomography before surgery. The patient underwent a bilateral retroperitoneoscopy adenomectomy and glucocorticoid replacement therapy. Our case indicates that AVS is of great significance for obtaining information on the functional state of BAAs before surgery.