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Ectopic ACTH- and/or CRH-producing pheochromocytomas

Journal of Clinical Endocrinology and Metabolism Jul 29, 2020

Elliott PF, Berhane T, Ragnarsson O, et al. - In the present study, the researchers sought to review the features and outcomes of adrenocorticotropic hormone (ACTH)- and/or corticotrophin-releasing hormone (CRH)-secreting pheochromocytomas. PubMed/MEDLINE and Web of Science were systematically searched, identifying relevant reports published up to February 10, 2020. Ninety-one articles reporting on 99 cases of ACTH and/or CRH-secreting pheochromocytomas (CRH-secreting n = 4) were identified. At diagnosis, the median age was 49 years (IQR 38, 59.5) with a 2:1 female to male ratio. The majority of patients had Cushing syndrome, hypertension, and/or diabetes. In the vast majority of patients, blood pressure, glucose control, and biochemical parameters improved postoperatively. The most common complication was infections. In individuals presenting with ACTH-dependent Cushing syndrome and adrenal mass, ectopic ACTH- and/or CRH-secreting pheochromocytoma should be considered. Despite the challenge in the diagnosis, patient outcomes appear to be favorable.

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