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Title: Ectopic ACTH- and/or CRH-producing pheochromocytomas.
Authors: Elliott, Patrick F
Berhane, Thomas
Ragnarsson, Oskar
Falhammar, Henrik
Citation: © Endocrine Society 2020. All rights reserved. For permissions, please e-mail:
J Clin Endocrinol Metab. 2020 Jul 25:dgaa488. doi: 10.1210/clinem/dgaa488.
Abstract: CONTEXT: The characteristics of catecholamine-secreting pheochromocytomas have been well studied. However, less is known about the characteristics, management and outcome in patients with ectopic adrenocorticotropic hormone (ACTH) and/or corticotrophin releasing hormone (CRH)-secreting pheochromocytomas. OBJECTIVE: To review the characteristics and outcomes of ACTH- and/or CRH-secreting pheochromocytomas. DATA SOURCE: A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 February 2020. STUDY SELECTION: Original articles, including case reports and case series, reporting individual patient data from patients with ACTH and/or CRH-secreting pheochromocytomas. DATA EXTRACTION: Information on sex, age, symptoms at presentation, comorbidities, biochemistry, imaging, histopathology, and outcomes was extracted. DATA SYNTHESIS: We identified 91 articles reporting on 99 cases of ACTH and/or CRH-secreting pheochromocytomas (CRH-secreting n=4). Median age at diagnosis was 49 years (IQR 38, 59.5) with a 2:1 female to male ratio. Most patients presented with clinical Cushing syndrome (n = 79; 81%), hypertension (n = 87; 93%), and/or diabetes (n = 50; 54%). Blood pressure, glucose control, and biochemical parameters improved in the vast majority of patients postoperatively. Infections were the most common complication. Most cases (n=70, 88%) with reported long-term outcome survived to publication (median follow-up 6 months). CONCLUSION: Ectopic ACTH- and/or CRH-secreting pheochromocytoma should be considered in patients presenting with ACTH-dependent Cushing syndrome and adrenal mass. Despite the challenge in diagnosis, patient outcomes appear favourable.
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Journal title: The Journal of clinical endocrinology and metabolism
Publication Date: 2020-07-25
Type: Journal Article
DOI: 10.1210/clinem/dgaa488
Appears in Collections:(a) NT Health Research Collection

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