CASE REPORT article
Front. Endocrinol.
Sec. Neuroendocrine Science
Volume 16 - 2025 | doi: 10.3389/fendo.2025.1649633
Case report: Ectopic CRH Production by Adrenal Adenoma as a Unique Cause of ACTH-Dependent Cushing's Syndrome
Provisionally accepted- Medical University of Warsaw, Warsaw, Poland
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Isolated ectopic secretion of corticotropin-releasing hormone (CRH) is an exceedingly rare cause of Cushing's syndrome (CS), accounting for fewer than 1% of cases. Ectopic CS is an uncommon but potentially life-threatening condition that often necessitates urgent diagnostic evaluation and treatment.Hormonal testing may suggest a pituitary origin, complicating the diagnostic process. To date, isolated ectopic CRH production has primarily been reported in cases of medullary thyroid carcinoma, pheochromocytoma, and various neuroendocrine tumors. We present the first documented case of ACTHdependent CS resulting from CRH secretion by an adrenal cortical adenoma. A 68-year-old woman presented with severe hypercortisolemia and biochemically confirmed ACTH-dependent CS. Imaging revealed an unilateral adrenal mass without evidence of pituitary or extra-adrenal lesions. Dynamic endocrine testing was consistent with an ectopic source of ACTH. The patient underwent unilateral adrenalectomy, which led to full clinical and biochemical remission without prolonged adrenal insufficiency. Histopathological analysis confirmed an adrenal cortical adenoma showing focal immunoreactivity for CRH and absence of ACTH expression. This case highlights the importance of concidering ectopic CRH secretion in the differential diagnosis of atypical ACTH-dependent CS, especially in patients presenting with adrenal adenomas.
Keywords: Cushing's syndrome, ectopic CRH secretion, Adrenal adenoma, case report, ectopic Cushing`s syndrome
Received: 18 Jun 2025; Accepted: 04 Aug 2025.
Copyright: © 2025 Pokrzywa, Pachucki, Bobrowicz, Koperski and Ambroziak. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Agata Pokrzywa, Medical University of Warsaw, Warsaw, Poland
Urszula Ambroziak, Medical University of Warsaw, Warsaw, Poland
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