부산대학교 도서관

Introduction: In this report, we present a case of Ectopic Cushing's syndrome in a patient without any detectable extrapituitary tumor secreting adrenocorticotropic hormone (ACTH). The literature reports only a few cases of this rare condition, with the tumor site typically being identified subsequently. Additionally, we conducted a mini-review of the literature.Case report: Our patient presented with lower extremity edema, weight gain, uncontrolled hypertension, hypokalemic metabolic alkalosis, and hyperglycemia, all of which pointed to ACTH-dependent Cushing's syndrome. Despite extensive testing, we were unable to locate the source of ACTH secretion. The patient underwent a bilateral adrenalectomy due to the development of life-threatening hypercortisolism. However, no ACTH-secreting tumor was found even after a 12-month follow-up.Conclusion: Ectopic Cushing's syndrome is a rare and challenging condition that is often not diagnosed despite the use of advanced techniques. In cases of occult Cushing's syndrome, bilateral adrenalectomy should be considered when life-threatening hypercortisolism develops. The ACTH-secreting tumor may remain occult for years after the onset of symptoms, and patients with occult ectopic Cushing's syndrome undergoing bilateral adrenalectomy should receive prolonged follow-up for life. Further studies are necessary to improve the management of this complex condition.