Ectopic Cushing’s syndrome: still much more to understand. A case report and a mini-review

Abstract

Introduction-We describe the case of a patient Ectopic Cushing’s syndrome without founding any extrapituitary tumour secreting adrenocorticotropic hormone (ACTH). In the literature there are only a few reported cases and generally the site of the tumor is subsequently identified. In addiction we performed a mini-review of the literature. Case report-We report the case of a patient with lower extremity edema, weight gain, uncontrolled hypertension, hypokalemic metabolic alkalosis and hyperglycemia suffering from ACTH-dependent Cushing's syndrome. None of the instrumental examinations allowed us to locate the source of ACTH secretion. Despite a medical therapy with maximum dosage of steroidogenesis inhibitors a bilateral adrenalectomy was performed due to the development of a life-threatening hypercortisolism. After 12 months from surgery follow-up is still negative for ACTH-secreting tumour. Conclusion- In a lot of cases Cushing’s syndrome is still nowadays not diagnosed although current advanced techniques. In occult Cushing’s syndrome bilateral adrenalectomy is the best therapy when a life-threatening hypercortisolism develops. The ACTH-secreting tumour could be occult for many years after the onset of symptoms. Follow-up in patients with occult ectopic Cushing’s syndrome undergoing bilateral adrenalectomy should be prolonged for life