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Ectopic Cushing's syndrome due to retroperitoneal ACTH-producing paragangliomas
Fan Chen1, Xiangyu Wang2, Yang Wang3
1Department of Urology, Qilu Hospital, Shandong University, Jinan, Shandong, China;; College of Medicine, University of Kentucky, Lexington, KY, United States.
Abstract:
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup.
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