Role of Adrenalectomy in Ectopic ACTH Syndrome

Abstract

Evaluation of adrenalectomy in patients diagnosed with ectopic ACTH syndrome was studied. Twenty-three clinical cases diagnosed with ectopic ACTH syndrome were analyzed at Chinese Academy of Medical Sciences and Peking Union Medical College Hospital (PUMCH). Cases consisted of 14 males and 9 females, with mean age of 38 years. All 23 cases had positive clinical, biochemical and radiology evidence for diagnosis of Cushing's syndrome. Sixteen of the 23 cases were treated with total adrenalectomy and the remaining 7 were treated without surgical intervention. Sixteen cases, having no identifiable source of ectopic hormone production, experienced resolution of presenting signs and symptoms after undergoing bilateral or unilateral total adrenalectomy; 1-year survival was 67%, 2-year survival 41% and 5-year survival 15%. In patients treated conservatively without surgical intervention, 1-year survival was 0%. In patients with no identifiable source of ectopic hormone production, bilateral adrenalectomy followed by hormone replacement treatment is effective.