title: Surgical treatment of ectopic adrenocorticotropic hormone syndrome with intra-thoracic tumor.
abstract: BACKGROUND
  The study was to review the clinical manifestations and laboratory examinations of ectopic adrenocorticotropic hormone (ACTH) syndrome, and to analyze the efficacy of surgical treatment.
  
  
  METHODS
  The clinical data, surgical therapy, and outcome of 23 cases of ectopic ACTH syndrome accompanied by intra-thoracic tumors were reviewed. The tumors were removed from all the patients according to the principles of radical resection.
  
  
  RESULTS
  The tumors were confirmed as associated with ectopic ACTH secretion in 19 cases. Hyperglycemia and hypokalemia were recovered, while plasma cortisol, plasma ACTH and 24-hour urinary free cortisol (UFC) levels were significantly reduced after surgery in these 19 cases. Recurrences of the disease were found in six cases during following-up, and five of them died.
  
  
  CONCLUSIONS
  The thoracic cavity should be a focus in routine examinations of patients with symptoms of Cushing's syndrome (CS), because ectopic ACTH-producing tumors are commonly found in bronchus/lung and mediastinum. Despite the incidence of the pulmonary nodule secondary to opportunistic infection in some cases, surgery is still the first choice if the tumor is localized. The surgical procedure should be performed according to the principles in resection of lung cancer and mediastinal tumor. The surgical efficacy is significant for short-term periods; however, the recurrence of the disease in long-term periods is in great part related to distal metastasis or relapse of the tumor.
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Xiang
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Junbiao
authors.initials: J
authors.lastname: Hang
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Jiaming
authors.initials: J
authors.lastname: Che
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Zhongyuan
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Weicheng
authors.initials: W
authors.lastname: Qiu
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Ren
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Xiaoqing
authors.initials: X
authors.lastname: Yang
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Xiang
authors.affiliation: Department of Thoracic Surgery, Shanghai Jiaotong University Medical School affiliated Ruijin Hospital, Shanghai 200025, China.
authors.forename: Hecheng
authors.initials: H
authors.lastname: Li
keywords: Ectopic ACTH syndrome; bronchopulmonary carcinoid; surgical treatment; thymic neuroendocrine tumor