Endocrine Abstracts

Objective: Ectopic adrenocorticotropic hormone (ACTH-ectopic) syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze the clinical, biochemical, and radiological features, management, and treatment outcome of patients with EAS.

Methods: It was a retrospective case-record study of 47 patients with EAS. Clinical, biochemical, and radiological features and response to therapy and survival rate were measured.

Results: The median follow-up was 7 years. (range, 1–13 years.). None of the dynamic tests achieved 100% accuracy. Imaging correctly identified the lesion at first investigation in 80.9% of cases. Bronchial carcinoid tumors were the most common cause of EAS (n=27; 57.5%), followed by other neuroendocrine tumors (n=11, 23.4%). In 19.1% (9) of patients, the source of EAS was never found. Tumor histology and the presence of distant metastases were the main predictors of overall survival (P < 0.05). It is interesting that 40 patients (85,1%) have steroid osteoporosis. 10 patients were treated with calcium and vitamin D, 35 patients by bisphosphonates, 2 patients by denosumab and 3 by teriparatide.

Conclusions: ACTH-ectopic syndrome is a very rare condition with severe complications. There is no significant data and guidelines for osteoporosis treatment in such disease. So we need to improve our to improve the quality of life of such difficult patients.