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Case Reports
. 2021 Jun 24:2021:5593920.
doi: 10.1155/2021/5593920. eCollection 2021.

Ectopic Cushing's Syndrome Secondary to Metastatic Paraganglioma

R Daya  1   2 , C Wingfield  1 , P Sotshononda  1 , F Seedat  1   2 , S Bulbulia  1   2 , M D Simmons  3 , M Louw  3 , Z Bayat  1   2
Affiliations
Case Reports

Ectopic Cushing's Syndrome Secondary to Metastatic Paraganglioma

R Daya et al. Case Rep Endocrinol. .

Abstract

Paraneoplastic or ectopic Cushing's syndrome (CS) is a rare cause of endogenous hypercortisolism. It is due to ectopic adrenocorticotropic hormone (ACTH) secretion and has been reported in association with a variety of neuroendocrine tumors such as small-cell lung carcinoma, carcinoid tumors, and medullary carcinoma of the thyroid. Paragangliomas (PGLs) are rare neuroendocrine tumors that can secrete catecholamines. Case reports and reports of ectopic ACTH secretion from metastatic PGLs causing CS are exceedingly rare. We present a case of a 38-year-old female, who presented with typical signs, symptoms, and complications of CS, secondary to a PGL with widespread metastases, which eventually led to her demise.

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Conflict of interest statement

The authors report no conflicts of interest.

Figures

Figure 1
Figure 1
Contrast-enhanced computed tomography (CT) of the chest showing a lung mass measuring 4.4 × 4.0 × 4.0 cm.
Figure 2
Figure 2
CT of the abdomen showing multiple irregular lesions in the liver, with the largest measuring 6.0 × 6.0 cm (white arrow).
Figure 3
Figure 3
Haematoxylin-and-eosin- (H&E-) stained section of the liver showing a characteristic nesting pattern (“Zellballen”) of cells (400× magnification).
Figure 4
Figure 4
Photomicrograph (100×) of the liver showing strong diffuse staining of tumor cells with a chromogranin.
See this image and copyright information in PMC

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