Management of ectopic cushing

Gordan Vukman; Mirsala Solak; Polovina Tanja Skoric; Annemarie Balasko; Anela Novak; Marija Tripolski; Darko Kastelan; Ivana Kraljevic

doi:10.1530/endoabs.110.P512

P512

Prev Next

Section Contents Cite

Endocrine Abstracts (2025) 110 P512 | DOI: 10.1530/endoabs.110.P512

ECEESPE2025 Poster Presentations Endocrine Related Cancer (76 abstracts)

Management of ectopic cushing’s syndrome: a retrospective analysis of therapeutic outcomes

Gordan Vukman ¹ , Mirsala Solak ² , Tanja Skoric Polovina ² , Annemarie Balasko ² , Anela Novak ³ , Marija Tripolski ⁴ , Darko Kastelan ¹ , 2 & Ivana Kraljevic ¹ & 2

Author affiliations

¹School of Medicine, University of Zagreb, Division of Endocrinology, Department of Internal Medicine, University Hospital Centre Zagreb, Zagreb, Croatia; ²Division of Endocrinology, Department of Internal Medicine, University Hospital Centre Zagreb, Zagreb, Croatia; ³Division of Endocrinology, Department of Internal Medicine, University Hospital Centre Split, Split, Croatia; ⁴Polyclinic Borzan, Osijek, Croatia

JOINT2483

Background: Ectopic Cushing’s syndrome (ECS) is a rare and severe endocrine disorder caused by excessive ACTH secretion from extra-pituitary tumors. Given its high morbidity and mortality, timely diagnosis and effective management are crucial. This study evaluates the outcomes of pharmacological and surgical interventions in patients with confirmed ECS.

Methods: We conducted a single-center retrospective study analyzing ECS treatment outcomes from 2012 to 2024 at the Division of Endocrinology, University Hospital Centre Zagreb. Patients received pharmacological therapy (metyrapone, etomidate, ketoconazole) and/or underwent bilateral adrenalectomy. Descriptive statistical methods were used for data analysis.

Results: The study included 12 patients (median age: 43. 5 years; range: 22-74), with a female predominance (83. 3%). Identified ACTH-secreting tumors included lung carcinoid (n = 1), small cell lung carcinoma (n = 2), pancreatic neuroendocrine tumor (n = 3), medullary thyroid carcinoma (n = 1), breast neuroendocrine carcinoma (n = 1), thymus neuroendocrine carcinoma (n = 1), mediastinal neuroendocrine carcinoma (n = 1), and two cases with unknown tumor origin. At diagnosis, metastases were present in nine patients (75%). Pharmacological therapy was administered to seven patients (58. 3%) using metyrapone alone (n = 2), ketoconazole alone (n = 2), an etomidate-metyrapone combination (n = 1), or a metyrapone-ketoconazole combination (n = 2). The median dose of metyrapone used was 1500 (1000-4000) mg and ketoconazole 400 (200-800) mg. ECS was successfully controlled in three of these patients, while the remaining four required subsequent bilateral adrenalectomy. In total, nine patients (75%) underwent bilateral adrenalectomy, including five for whom surgery was the primary treatment. One patient achieved remission through lung resection. At the time of analysis, seven patients had died due to disease progression, with a median time to death of 16 (1–51) months. The median overall survival was 20 (1–154) months.

Conclusion: The management of ECS requires a multimodal approach, incorporating both pharmacological and surgical interventions. While pharmacological therapy was effective in some patients, bilateral adrenalectomy was necessary in certain cases