1. 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
- Author
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Chen YY, Li SQ, Liu HS, Qin YZ, Li L, Huang C, Bi YL, Meng YX, He J, Zhou XY, and Ma DJ
- Subjects
thymus gland ,ACTH syndrome ,ectopic ,neuroendocrine tumors ,lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,lcsh:RC254-282 ,surgical procedures - Abstract
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 Ma11Department 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 ChinaBackground 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 32.5years (range: 13–47years), and the median disease duration was 8.5months (range: 1–150months). 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 2weeks after surgery. No other morbidity or mortality occurred during the perioperative period. The median overall survival (OS) was 41months (95% CI: 30.3–51.7months), and the progression-free survival was 28months (95% CI:21.6–34.3months). 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
- Published
- 2016