The Ectopic Adrenocorticotropin Syndrome: Clinical Features, Diagnosis, Management, and Long-Term Follow-Up

Context: There are few large series of patients with ectopic, nonpituitary, corticotropin (ACTH) secretion (EAS). Objective: The objective of this study was to analyze the clinical, biochemical, and radiological features, management, and treatment outcome of patients with EAS. Design: This was a retrospective case-record study. Setting: The setting for this study was a tertiary referral hospital center. Patients: Forty patients with EAS were studied. Main Outcome Measures: Clinical, biochemical, and radiological features and response to therapy and survival were measured. Results: The median follow-up was 5 yr (range, 2–30 yr). None of the dynamic tests achieved 100% accuracy, but bilateral inferior petrosal sinus sampling showed an absent central gradient in all but one case (one of 12). Imaging correctly identified the lesion at first investigation in 65% of cases. Bronchial carcinoid tumors were the most common cause of EAS (n = 12; 30%), followed by other neuroendocrine tumors (n = 13, 32.5%). In 12.5...