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Ectopic adrenocorticotropic hormone syndrome caused by neuroendocrine tumors of the thymus: 30-year experience with 16 patients at a single institute in the People’s Republic of China

Authors Chen Y, Li S, Liu H, Qin Y, Li L, Huang C, Bi Y, Meng Y, He J, Zhou X, Ma D

Received 15 November 2015

Accepted for publication 2 February 2016

Published 26 April 2016 Volume 2016:9 Pages 2193—2201

DOI https://doi.org/10.2147/OTT.S100585

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Wei An

Peer reviewer comments 2

Editor who approved publication: Professor Min Li


Ye-ye Chen,1 Shan-qing Li,1,2 Hong-sheng Liu,1,2 Ying-zhi Qin,1 Li Li,1 Cheng Huang,1 Ya-lan Bi,3 Yun-xiao Meng,3 Jia He,1 Xiao-yun Zhou,1 Dong-jie Ma1

1Department of Thoracic Surgery, 2Key Laboratory of Endocrinology, 3Department of Pathology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, People’s Republic of China

Background and purpose: Thymic neuroendocrine carcinomas (TNECs) are extremely uncommon. Certain cases of TNECs can produce the adrenocorticotropic hormone (ACTH) and cause ectopic ACTH syndrome (EAS). The current literature on this topic consists mainly of case reports, and therapeutic guidelines are lacking. The aim of this study was to discuss the diagnosis, surgical management, and prognosis of EAS caused by TNECs to improve clinical experience with this rare disease.
Methods: From June 1984 to June 2014, at the Peking Union Medical College Hospital, the surgical interventions and follow-up outcomes of 16 consecutive patients (eight men and eight women) with EAS caused by TNECs were retrospectively analyzed.
Results: The median age was 32.5 years (range: 13–47 years), and the median disease duration was 8.5 months (range: 1–150 months). All patients presented with clinical and biochemical evidence indicating a diagnosis of Cushing’s syndrome. Contrast-enhanced thoracic computed tomography scans were critical to locating the ACTH-producing tumor and evaluating the feasibility of resection. All patients underwent surgery. One patient died of septicemia in the intensive care unit 2 weeks after surgery. No other morbidity or mortality occurred during the perioperative period. The median overall survival (OS) was 41 months (95% CI: 30.3–51.7 months), and the progression-free survival was 28 months (95% CI: 21.6–34.3 months). Both overall survival (P=0.002) and progression-free survival (P=0.030) improved significantly after complete resection.
Conclusion: TNEC is an extremely aggressive disease that should be considered when treating patients with Cushing’s syndrome due to ectopic ACTH secretion. In particular, all suspected patients should undergo contrast-enhanced thoracic computed tomography scans to facilitate early diagnosis. The current first-line treatment is surgical resection, and complete resection is a favorable prognostic factor. However, additional patients and a longer follow-up will be needed to determine the variables that are predictive of survival and to improve patient prognosis.

Keywords: ACTH syndrome, ectopic, neuroendocrine tumors, thymus gland, surgical procedures

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