Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Sep-Oct;4(5):e375-e378.
doi: 10.4158/ACCR-2018-0045. Epub 2018 May 1.

THYMIC CARCINOID WITH ADRENOCORTICOTROPIC HORMONE-PRODUCING ECTOPIC CUSHING SYNDROME AND EMPTY SELLA

Artak Labadzhyan  1 , Se-Min Kim  1 , Jane Rhyu  1 , Takako Araki  2 , Ali Mahtabifard  3 , Shlomo Melmed  1
Affiliations

THYMIC CARCINOID WITH ADRENOCORTICOTROPIC HORMONE-PRODUCING ECTOPIC CUSHING SYNDROME AND EMPTY SELLA

Artak Labadzhyan et al. AACE Clin Case Rep. 2018 Sep-Oct.

Abstract

Objective: Ectopic Cushing's 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's syndrome secondary to a large adrenocorticotropic hormone (ACTH) producing thymic carcinoid in a patient with history of primary hyperaldosteronism.

Results: A 43-year-old female with a 20-year history of an aldosterone-secreting adrenocortical adenoma status post right adrenalectomy presented with acute onset of proximal muscle weakness, swelling, facial hirsutism, and severe hypokalemia. Ectopic Cushing's Syndrome was suspected based on the sudden symptom onset and markedly elevated 24-hr urine cortisol and ACTH levels. MRI revealed an empty pituitary sella and a large (7.3 cm) mediastinal mass visible on chest CT. 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: The case highlights clinical features, challenges in diagnostic work up, treatment modalities, and associated endocrine findings in a thymic carcinoid abutting the heart and presenting with ectopic ACTH secretion.

Keywords: ACTH; EAS; Thymic carcinoid; ectopic Cushing’s syndrome; empty sella; hyperaldosteronism; neuroendocrine carcinoma of thymus.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Left panel: magnetic resonance imaging (MRI) pituitary with empty sella (red arrow). Right panel: computer tomography (CT) chest with 7.3 cm mediastinal mass (red arrow).
Figure 2.
Figure 2.
Left panel: Diffusively positive ACTH staining of tissue from mediastinal mass. Right panel: Positive immunostaining of isolated cell culture. Green: ACTH; blue: DAPI (4',6-diamidino-2-phenylindole).
See this image and copyright information in PMC

References

    1. Ilias I, Torpy DJ, Pacak K, Mullen N, Wesley RA, Nieman LK. Cushing’s syndrome due to ectopic corticotropin secretion: twenty years’ experience at the National Institutes of Health. J Clin Endocrinol Metab. 2005;90:4955–4962. - PubMed
    1. Isidori AM, Kaltsas GA, Pozza C, et al. The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up. J Clin Endocrinol Metab. 2006;91:371–377. - PubMed
    1. Aniszewski JP, Young WF Jr., Thompson GB, Grant CS, van Heerden JA. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion. World J Surg 2001;25:934–940. - PubMed
    1. Gaur P, Leary C, Yao JC. Thymic neuroendocrine tumors: a SEER database analysis of 160 patients. Ann Surgery. 2010;251:1117–1121. - PubMed
    1. Honda T, Nakamura T, Saito Y, Ohyama Y, Sumino H, Kurabayashi M. Combined primary aldosteronism and preclinical Cushing’s syndrome: an unusual case presentation of adrenal adenoma. Hypertens Res 2001;24:723–726. - PubMed

LinkOut - more resources