13
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

      review-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is rarely caused by a pheochromocytoma. We present a case of a 46-year-old woman who developed severe hypertension, hypokalemia, and typical Cushingoid features. Investigations revealed extremely high metanephrine, cortisol, and ACTH levels. Imaging showed a 3.8-cm left adrenal mass. Preoperative control of hypertension and hypokalemia was very challenging. The patient was cured after surgical removal of the adrenal mass. We followed this by a review of the literature using the databases Google Scholar and PubMed. A total of 58 cases have been reported to date. In summary, ACTH-producing pheochromocytoma is a rare condition that poses a clinical challenge in the perioperative period. It is important that physicians be aware of such a condition because early recognition and treatment are crucial to decrease morbidity and mortality.

          Abstract

          We present a case of ACTH-producing pheochromocytoma, a life-threatening condition if not recognized and treated early. We follow it with a review of all reported cases to improve physician awareness.

          Related collections

          Most cited references56

          • Record: found
          • Abstract: found
          • Article: not found

          Cushing's syndrome.

          Cushing's syndrome results from lengthy and inappropriate exposure to excessive glucocorticoids. Untreated, it has significant morbidity and mortality. The syndrome remains a challenge to diagnose and manage. Here, we review the current understanding of pathogenesis, clinical features, diagnostic, and differential diagnostic approaches. We provide diagnostic algorithms and recommendations for management.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Morbidity and mortality in Cushing's disease: an epidemiological approach.

            Although Cushing's disease is a well documented clinical entity, there is no epidemiological information about it. The present study tries to obtain this information. Forty-nine patients affected by Cushing's disease living in Vizcaya (Spain) between 1975 and 1992 were considered for an epidemiological study. The prevalence of known cases at the end of 1992 was 39.1 per million inhabitants. The average incidence of newly diagnosed cases was 2.4 cases per million people per year. Cushing's disease was more frequent in women (n = 46) than in men (n = 3), with a ratio of 15:1. Diabetes mellitus and hypertension were observed in 38.7 and 55.1% of patients, respectively. Remission of Cushing's disease was achieved in 36 out of 41 patients (87.5%). In general, the mortality was higher than that expected for the control population (standardized mortality ratio, SMR 3.8, 95% confidence interval, CI 2.5-17.9, P < 0.03). Concerning the cause of death, the SMR of vascular disease was 5 (95% CI 3.4-48.6, P < 0.05). Higher age, persistence of hypertension and abnormalities of glucose metabolism after treatment, were independent predictors of mortality (multivariate analyses, P < 0.01). Prevalence of Cushing's disease was 39.1 cases/million inhabitants and average incidence was 2.4 cases/million per year. Mortality was elevated, due to vascular disease, associated with higher age, persistence of hypertension and impaired glucose metabolism.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: found
              Is Open Access

              Cushing’s syndrome: epidemiology and developments in disease management

              Cushing’s syndrome is a rare disorder resulting from prolonged exposure to excess glucocorticoids. Early diagnosis and treatment of Cushing’s syndrome is associated with a decrease in morbidity and mortality. Clinical presentation can be highly variable, and establishing the diagnosis can often be difficult. Surgery (resection of the pituitary or ectopic source of adrenocorticotropic hormone, or unilateral or bilateral adrenalectomy) remains the optimal treatment in all forms of Cushing’s syndrome, but may not always lead to remission. Medical therapy (steroidogenesis inhibitors, agents that decrease adrenocorticotropic hormone levels or glucocorticoid receptor antagonists) and pituitary radiotherapy may be needed as an adjunct. A multidisciplinary approach, long-term follow-up, and treatment modalities customized to each individual are essential for optimal control of hypercortisolemia and management of comorbidities.
                Bookmark

                Author and article information

                Contributors
                Journal
                J Endocr Soc
                J Endocr Soc
                jes
                Journal of the Endocrine Society
                Endocrine Society (Washington, DC )
                2472-1972
                01 July 2018
                24 May 2018
                : 2
                : 7
                : 621-630
                Affiliations
                [1 ]Division of Endocrinology and Metabolism, Internal Medicine Department, John C. Edwards School of Medicine, Marshall University, Huntington, West Virginia
                [2 ]Department of Internal Medicine, John C. Edwards School of Medicine, Marshall University, Huntington, West Virginia
                [3 ]Department of Cardiovascular Disease, John C. Edwards School of Medicine, Marshall University, Huntington, West Virginia
                Author notes
                Correspondence:  Jenan N. Gabi, MD, 1249 15th street, Huntington, West Virginia 25701. E-mail: gabij@ 123456live.marshall.edu .
                Article
                js_201800086
                10.1210/js.2018-00086
                6007244
                29942926
                9bcbf16d-cdfc-4c8b-b2ac-ca2577c33589
                Copyright © 2018 Endocrine Society

                This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 28 March 2018
                : 21 May 2018
                Page count
                Pages: 10
                Categories
                Mini-Reviews
                Adrenal

                acth-producing pheochromocytoma,cushing syndrome,ectopic,pheochromocytoma

                Comments

                Comment on this article