ORIGINAL RESEARCH article
Front. Endocrinol.
Sec. Neuroendocrine Science
This article is part of the Research TopicUnveiling the Complexity of Neuroendocrine Neoplasms: Biomarkers, Classification, and Translational PerspectivesView all 6 articles
Case Report: Ectopic Cushing's syndrome caused by a pancreatic neuroendocrine tumor managed through dual steroidogenesis inhibition and endoscopic ultrasound-guided ethanol ablation
Provisionally accepted
Łukasz Krzystek1
Karol Piotr Sagan2*Elżbieta Andrysiak Mamos2Szymon Retfiński1Karolina Buć1Wiktoria Marczak1Anna Brzeska3Martyna Patalong-Wójcik3Ewa Żochowska3Krzysztof Dąbkowski4
Anhelli Syrenicz3- 1Pomeranian Medical University, Szczecin, Poland
- 2Department of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University, Szczecin, Poland
- 3Department of Endocrinology, Metabolic and Internal Diasease, Pomeranian Medical University, Szczecin, Poland
- 4Department of Gastroenterology, Pomeranian Medical University, Szczecin, Poland
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Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (EAS) is a rare complication of neuroendocrine tumors (NETs). Severe hypercortisolism (SH) requires urgent medical intervention due to its life-threatening consequences. We report a 74-year-old female patient with an ACTH-secreting pancreatic NET (pNET) who presented with rapidly progressive cognitive decline, muscle weakness, severe hypokalemia, and hyperglycemia. Laboratory evaluation confirmed ACTH-dependent Cushing's syndrome with loss of diurnal cortisol rhythm and panhypopituitarism. Surgical treatment was contraindicated because of significant comorbidities. The initial management included intravenous etomidate infusion. Subsequently, osilodrostat was introduced as long-term oral therapy. Marked clinical and hormonal improvements were observed, including the normalization of potassium and cortisol levels, resolution of neuropsychiatric symptoms, and restoration of mobility. After 19 months of osilodrostat therapy, endoscopic ultrasound-guided ethanol ablation of the pancreatic lesion was performed, and medical therapy was discontinued. This case demonstrates the effectiveness of dual steroidogenesis blockade with etomidate and osilodrostat in both the acute and chronic management of severe ectopic Cushing's syndrome due to pNET. It also highlights the role of endoscopic ethanol ablation as a minimally invasive curative option for patients who are unfit for surgery.
Keywords: Ectopic Cushing's syndrome, Pancreatic neuroendocrine tumor, ACTH, Hypercortisolism, Osilodrostat, Etomidate, Ethanol ablation
Received: 06 Sep 2025; Accepted: 27 Nov 2025.
Copyright: © 2025 Krzystek, Sagan, Andrysiak Mamos, Retfiński, Buć, Marczak, Brzeska, Patalong-Wójcik, Żochowska, Dąbkowski and Syrenicz. 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: Karol Piotr Sagan
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