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Ectopic ACTH- and/o...
Ectopic ACTH- and/or CRH-Producing Pheochromocytomas
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Elliott, P. F. (author)
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Berhane, T. (author)
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- Ragnarsson, Oskar, 1971 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin, avdelningen för invärtesmedicin och klinisk nutrition,Institute of Medicine, Department of Internal Medicine and Clinical Nutrition
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- Falhammar, H. (author)
- Karolinska Institutet
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(creator_code:org_t)
- 2020-07-25
- 2021
- English.
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In: Journal of Clinical Endocrinology & Metabolism. - : The Endocrine Society. - 0021-972X .- 1945-7197. ; 106:2, s. 598-608
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Abstract
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- 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 (interquartile range 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 favorable.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine (hsv//eng)
Keyword
- adrenal mass
- Cushing syndrome
- hypertension
- diabetes
- hypokalemia
- mortality
- Endocrinology & Metabolism
Publication and Content Type
- ref (subject category)
- art (subject category)
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