Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella

ABSTRACT: Objective: Ectopic Cushing syndrome secondary to thymic carcinoid is a rare disorder that may be difficult to diagnose and manage.Methods: We describe a case of severe Cushing syndrome secondary to a large, adrenocorticotropic hormone (ACTH)-producing thymic carcinoid in a patient with a h...

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Main Authors: Artak Labadzhyan, MD, Se-Min Kim, MD, Jane Rhyu, MD, Takako Araki, MD, Ali Mahtabifard, MD, Shlomo Melmed, MD
Format: Article
Language:English
Published: Elsevier 2018-09-01
Series:AACE Clinical Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2376060520301590
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spelling doaj-e301188bf5db4118b3782a3eecb2b4c02021-04-30T07:23:45ZengElsevierAACE Clinical Case Reports2376-06052018-09-0145e375e378Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty SellaArtak Labadzhyan, MD0Se-Min Kim, MD1Jane Rhyu, MD2Takako Araki, MD3Ali Mahtabifard, MD4Shlomo Melmed, MD5From the Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, California; Address correspondence to Dr. Artak Labadzhyan, Cedars-Sinai Medical Center, 127 South San Vicente Boulevard, Suite A6600, Los Angeles, CA 90048.From the Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CaliforniaFrom the Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CaliforniaDivision of Endocrinology, Diabetes and Metabolism, Department of Medicine, University of Minnesota, Minneapolis, MinnesotaDivision of Thoracic Surgery, Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, California.From the Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CaliforniaABSTRACT: Objective: Ectopic Cushing syndrome secondary to thymic carcinoid is a rare disorder that may be difficult to diagnose and manage.Methods: We describe a case of severe Cushing syndrome secondary to a large, adrenocorticotropic hormone (ACTH)-producing thymic carcinoid in a patient with a history of primary hyperaldosteronism.Results: A 43-year-old female with a 20-year history of an aldosterone-secreting adrenocortical adenoma following a right adrenalectomy presented with acute onset of proximal muscle weakness, swelling, facial hirsutism, and severe hypokalemia. Ectopic Cushing syndrome was suspected based on the sudden symptom onset and markedly elevated 24-hour urine cortisol and ACTH levels. Magnetic resonance imaging revealed an empty pituitary sella and a large (7.3 cm) mediastinal mass visible on chest computed tomography. The mass was resected by video-assisted thoracoscopic surgery, resulting in resolution of symptoms and cortisol levels. Pathology assessment confirmed well-differentiated thymic carcinoid with positive ACTH staining.Conclusion: This case highlights the clinical features, challenges in diagnostic work up, treatment modalities, and associated endocrine findings in a thymic carcinoid abutting the heart in a patient presenting with ectopic ACTH secretion.Abbreviations: ACTH = adrenocorticotropic hormone;EAS = ectopic adrenocorticotropic hormone syndrome;MRI = magnetic resonance imaging;VATS = video-assisted thoracoscopic surgeryhttp://www.sciencedirect.com/science/article/pii/S2376060520301590
collection DOAJ
language English
format Article
sources DOAJ
author Artak Labadzhyan, MD
Se-Min Kim, MD
Jane Rhyu, MD
Takako Araki, MD
Ali Mahtabifard, MD
Shlomo Melmed, MD
spellingShingle Artak Labadzhyan, MD
Se-Min Kim, MD
Jane Rhyu, MD
Takako Araki, MD
Ali Mahtabifard, MD
Shlomo Melmed, MD
Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
AACE Clinical Case Reports
author_facet Artak Labadzhyan, MD
Se-Min Kim, MD
Jane Rhyu, MD
Takako Araki, MD
Ali Mahtabifard, MD
Shlomo Melmed, MD
author_sort Artak Labadzhyan, MD
title Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
title_short Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
title_full Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
title_fullStr Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
title_full_unstemmed Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella
title_sort thymic carcinoid with adrenocorticotropic hormone-producing ectopic cushing syndrome and empty sella
publisher Elsevier
series AACE Clinical Case Reports
issn 2376-0605
publishDate 2018-09-01
description ABSTRACT: Objective: Ectopic Cushing syndrome secondary to thymic carcinoid is a rare disorder that may be difficult to diagnose and manage.Methods: We describe a case of severe Cushing syndrome secondary to a large, adrenocorticotropic hormone (ACTH)-producing thymic carcinoid in a patient with a history of primary hyperaldosteronism.Results: A 43-year-old female with a 20-year history of an aldosterone-secreting adrenocortical adenoma following a right adrenalectomy presented with acute onset of proximal muscle weakness, swelling, facial hirsutism, and severe hypokalemia. Ectopic Cushing syndrome was suspected based on the sudden symptom onset and markedly elevated 24-hour urine cortisol and ACTH levels. Magnetic resonance imaging revealed an empty pituitary sella and a large (7.3 cm) mediastinal mass visible on chest computed tomography. The mass was resected by video-assisted thoracoscopic surgery, resulting in resolution of symptoms and cortisol levels. Pathology assessment confirmed well-differentiated thymic carcinoid with positive ACTH staining.Conclusion: This case highlights the clinical features, challenges in diagnostic work up, treatment modalities, and associated endocrine findings in a thymic carcinoid abutting the heart in a patient presenting with ectopic ACTH secretion.Abbreviations: ACTH = adrenocorticotropic hormone;EAS = ectopic adrenocorticotropic hormone syndrome;MRI = magnetic resonance imaging;VATS = video-assisted thoracoscopic surgery
url http://www.sciencedirect.com/science/article/pii/S2376060520301590
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