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      A case of adrenal Cushing’s syndrome with bilateral adrenal masses

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          Summary

          A functional lesion in corticotrophin (ACTH)-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol ( 131I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL). No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.

          Learning points

          • The clinical presentation of Cushing’ syndrome includes symptoms and signs of fat redistribution and protein-wasting features.

          • The diagnosis of patients with ACTH-independent Cushing’s syndrome with bilateral adrenal masses is challenging for localisation of the lesion.

          • Both adrenal venous sampling and 131I-NP-59 scintigraphy are good methods to use in these patients with Cushing’s syndrome presenting with bilateral adrenal masses.

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          Most cited references13

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          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.
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            Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline.

            Our objective was to develop clinical practice guidelines for the diagnosis and treatment of patients with primary aldosteronism. The Task Force comprised a chair, selected by the Clinical Guidelines Subcommittee (CGS) of The Endocrine Society, six additional experts, one methodologist, and a medical writer. The Task Force received no corporate funding or remuneration. Systematic reviews of available evidence were used to formulate the key treatment and prevention recommendations. We used the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) group criteria to describe both the quality of evidence and the strength of recommendations. We used "recommend" for strong recommendations and "suggest" for weak recommendations. Consensus was guided by systematic reviews of evidence and discussions during one group meeting, several conference calls, and multiple e-mail communications. The drafts prepared by the task force with the help of a medical writer were reviewed successively by The Endocrine Society's CGS, Clinical Affairs Core Committee (CACC), and Council. The version approved by the CGS and CACC was placed on The Endocrine Society's Web site for comments by members. At each stage of review, the Task Force received written comments and incorporated needed changes. We recommend case detection of primary aldosteronism be sought in higher risk groups of hypertensive patients and those with hypokalemia by determining the aldosterone-renin ratio under standard conditions and that the condition be confirmed/excluded by one of four commonly used confirmatory tests. We recommend that all patients with primary aldosteronism undergo adrenal computed tomography as the initial study in subtype testing and to exclude adrenocortical carcinoma. We recommend the presence of a unilateral form of primary aldosteronism should be established/excluded by bilateral adrenal venous sampling by an experienced radiologist and, where present, optimally treated by laparoscopic adrenalectomy. We recommend that patients with bilateral adrenal hyperplasia, or those unsuitable for surgery, optimally be treated medically by mineralocorticoid receptor antagonists.
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              Adrenal vein sampling: how to make it quick, easy, and successful.

              Adrenal vein sampling has a reputation as a difficult procedure. However, it is being performed more frequently at some institutions due to the realization that primary aldosteronism is more common than previously believed. At the author's institution, adrenal vein sampling with computed tomographic (CT) and laboratory correlation has been performed more than 800 times in the past 10 years. Adrenal vein sampling is used to determine whether autonomous hormone production is unilateral or bilateral; unilateral secretion can be treated with surgery. The venous drainage of each adrenal gland is predominantly via a central vein. Recognition of the right adrenal vein is the crux of adrenal vein sampling. CT is useful in planning adrenal vein sampling by demonstrating the anatomy and positions of the adrenal veins. A small amount of contrast material is injected gently and slowly into the adrenal vein; it is not necessary to perform formal venography to outline the entire gland. To confirm that the vein is draining the majority of adrenal cortical blood, the adrenal vein sample should have a significantly higher level of cortisol than a peripheral sample. Adrenal glands that are producing aldosterone demonstrate an aldosterone-cortisol ratio that is higher than the peripheral value. Copyright RSNA, 2005.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                edm
                EDM Case Reports
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                1 May 2016
                2016
                : 2016
                : 150118
                Affiliations
                [1 ]Department of Medicine, Taipei City Hospital , Zhongxing Branch, Taipei, Taiwan
                [2 ]Department of Medicine, Division of Endocrinology and Metabolism, Taipei Veterans General Hospital , Taipei, Taiwan
                [3 ]Faculty of Medicine, National Yang-Ming University School of Medicine , Taipei, Taiwan
                [4 ]Department of Otorhinolaryngology-Head and Neck Surgery, Division of Otology, Taipei Veterans General Hospital , Taipei, Taiwan
                Author notes
                Correspondence should be addressed to Liang-Yu Lin Email: linly@ 123456vghtpe.gov.tw
                Article
                EDM150118
                10.1530/EDM-15-0118
                4870494
                27252858
                3a4ee5ba-fe91-439a-9e3d-56a9a5af7738
                © 2016 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 20 March 2016
                : 18 April 2016
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

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