Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

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.

Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review

“…We also carefully retrieved additional references from case reports within the review literature that were not captured in the PubMed search procedure. Although most tumors (53 out of 67) originated from the adrenal glands, 14 cases of ACTH-secreting paragangliomas were found to arise from extra-adrenal sites, including the retroperitoneum [3][4][5][6], mediastinum [7][8][9], paranasal region [10][11][12], lung [13], thymus [14], and cervical and abdominal areas [15,16]. The ages of the patients ranged from 12 to 80 years old, with a mean age of 48.8 years (±14.5 years SD).…”

“…These findings imply that the potency of catecholamine and ACTH excretion are not interrelated. In the following analysis, we converted the terms "high ACTH" [8], "normal cortisol" [22], "high cortisol" [22], "cortisol values of x μg/dL or greater", and "normal urinary MN or NMN" [3,4,12,13,15,23] to numerical values of 70 pg/mL, 12 µg/dL, 22 µg/dL, 1.1 times (110%) of x μg/dL, and 0.3 mg/day, respectively. Additionally, we used the median values to illustrate the range of values and focused on the physical Cushingoid symptoms while excluding hypertension, diabetes, and biochemical changes.…”