ACTH-dependent Cushing’s syndrome

Summary

Introduction. Ectopic adrenocorticotropic hormone (ACTH) syndrome is very rare endocrine disorder caused by excess secretion of ACTH by non-pitutary tumor. Prolonged glucocorticoid excess causes Cushing’s syndrome, associated with complications, increasing mortality. First choice treatment is definitive tumor resection. Main issue in diagnosis is to find ectopic ACTH secretion source. For patients with occult or metastatic ectopic ACTH secretion adrenal steroidogenesis inhibitors are recommended as first line therapy to control hypercortisolemia.