Case Report: ACTH- and CRH-secreting pheochromocytoma as a very rare cause of ectopic Cushing syndrome

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) represents a rare clinical entity characterized by severe hypercortisolemia. Pheochromocytoma, a neuroendocrine neoplasm originating from chromaffin cells within the adrenal medulla, is itself an uncommon diagnosis. The phenomenon of ectopic hormone production by pheochromocytomas is exceedingly rare, occurring in less than 1% of cases. In most instances, hypercortisolemia associated with pheochromocytoma is attributed to aberrant ACTH secretion. This report presents an exceptional case of EAS caused by a pheochromocytoma exhibiting co-expression of both ACTH and corticotropin-releasing hormone (CRH).

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Journal
Frontiers in Endocrinology
Published
2025-11-17
DOI
https://doi.org/10.3389/fendo.2025.1580876
Primary Topic
Pituitary Gland Disorders and Treatments
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article
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article

Case Report: ACTH- and CRH-secreting pheochromocytoma as a very rare cause of ectopic Cushing syndrome

Rafał Czepczyński, Marek A. Ruchała, Nadia Sawicka‐Gutaj, Barbara Bromińska et al.
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article

Case Report: ACTH- and CRH-secreting pheochromocytoma as a very rare cause of ectopic Cushing syndrome

Rafał Czepczyński, Marek A. Ruchała, Nadia Sawicka‐Gutaj, Barbara Bromińska, Małgorzata Lange‐Ratajczak, Ewelina Szczepanek‐Parulska, Małgorzata Janicka‐Jedyńska, T Wierzbicki
article en

Abstract

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) represents a rare clinical entity characterized by severe hypercortisolemia. Pheochromocytoma, a neuroendocrine neoplasm originating from chromaffin cells within the adrenal medulla, is itself an uncommon diagnosis. The phenomenon of ectopic hormone production by pheochromocytomas is exceedingly rare, occurring in less than 1% of cases. In most instances, hypercortisolemia associated with pheochromocytoma is attributed to aberrant ACTH secretion. This report presents an exceptional case of EAS caused by a pheochromocytoma exhibiting co-expression of both ACTH and corticotropin-releasing hormone (CRH).

Frontiers in EndocrinologyVol. 16
Poznan University of Medical Sciences (PL)
Openalex Percentile: Top 54%
Pituitary Gland Disorders and Treatments
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