{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T10:01:15Z","timestamp":1788861675655,"version":"build-2803163510"},"reference-count":45,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T00:00:00Z","timestamp":1718323200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:p>Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%\u201310% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.<\/jats:p>","DOI":"10.3389\/fendo.2024.1399930","type":"journal-article","created":{"date-parts":[[2024,7,19]],"date-time":"2024-07-19T05:50:01Z","timestamp":1721368201000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Case report: A challenging case of severe Cushing\u2019s syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor"],"prefix":"10.3389","volume":"15","author":[{"given":"Lukasz","family":"Dzialach","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnieszka","family":"Wojciechowska-Luzniak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Maksymowicz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Przemys\u0142aw","family":"Witek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,6,14]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(14)61375-1","article-title":"Cushing\u2019s syndrome","volume":"386","author":"Lacroix","year":"2015","journal-title":"Lancet"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.2147\/CLEP.S44336","article-title":"Cushing's syndrome: epidemiology and developments in disease management","volume":"281","author":"Feelders","year":"2015","journal-title":"CLEP"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-17-0036","article-title":"Paraneoplastic endocrine syndromes","volume":"24","author":"Dimitriadis","year":"2017","journal-title":"Endocrine-Related Cancer"},{"key":"B4","article-title":"Ectopic Cushing\u2019s syndrome in light of modern diagnostic techniques and treatment options","volume":"36","author":"Witek","year":"2015","journal-title":"Neuro Endocrinol Lett"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-19-0877","article-title":"MANAGEMENT OF ENDOCRINE DISEASE: Cushing\u2019s syndrome due to ectopic ACTH secretion: an expert operational opinion","volume":"182","author":"Young","year":"2020","journal-title":"Eur J Endocrinol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"e0242679","DOI":"10.1371\/journal.pone.0242679","article-title":"Ectopic ACTH syndrome of different origin\u2014Diagnostic approach and clinical outcome","volume":"15","author":"Pale\u0144-Tytko","year":"2020","journal-title":"Exp One Clin Centre PloS One"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3181dd4ec4","article-title":"Thymic neuroendocrine tumors: A SEER database analysis of 160 patients","volume":"251","author":"Gaur","year":"2010","journal-title":"Ann Surg"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1159\/000472255","article-title":"Thymic neuroendocrine neoplasms: biological behaviour and therapy","volume":"105","author":"Jia","year":"2017","journal-title":"Neuroendocrinology"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s11154-021-09660-2","article-title":"Ectopic Cushing\u2019s syndrome due to thymic neuroendocrine tumours: a systematic review","volume":"22","author":"Guerrero-P\u00e9rez","year":"2021","journal-title":"Rev Endocr Metab Disord"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2011-3355","article-title":"Neuroendocrine ACTH-producing tumor of the thymus\u2014Experience with 12 patients over 25 years","volume":"97","author":"Neary","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"key":"B11","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1016\/j.jtcvs.2014.08.061","article-title":"Outcome of primary neuroendocrine tumors of the thymus: A joint analysis of the International Thymic Malignancy Interest Group and the European Society of Thoracic Surgeons databases","volume":"149","author":"Filosso","year":"2015","journal-title":"J Thorac Cardiovasc Surg"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1016\/j.thorsurg.2014.05.007","article-title":"Thymic neuroendocrine tumors","volume":"24","author":"Lausi","year":"2014","journal-title":"Thorac Surg Clinics"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-12-0274","article-title":"THERAPY IN ENDOCRINE DISEASE: Etomidate in the management of hypercortisolaemia in Cushing\u2019s syndrome: a review","volume":"167","author":"Preda","year":"2012","journal-title":"Eur J Endocrinol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1016\/j.beem.2021.101490","article-title":"Updates in adrenal steroidogenesis inhibitors for Cushing\u2019s syndrome \u2013 A practical guide","volume":"35","author":"Varlamov","year":"2021","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1155\/2020\/2025631","article-title":"An adrenocortical carcinoma associated with non-islet cell tumor hypoglycemia and aberrant ACTH production","volume":"2020","author":"Dilrukshi","year":"2020","journal-title":"Case Rep Endocrinol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.11100569","article-title":"Characterization of adrenal masses by using FDG PET: A systematic review and meta-analysis of diagnostic test performance","volume":"259","author":"Boland","year":"2011","journal-title":"Radiology"},{"key":"B17","doi-asserted-by":"publisher","first-page":"20170520","DOI":"10.1259\/bjr.20170520","article-title":"Diagnostic accuracy of 18 F-FDG PET or PET\/CT for the characterization of adrenal masses: a systematic review and meta-analysis","volume":"91","author":"Kim","year":"2018","journal-title":"BJR"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgad138","article-title":"The diagnostic value of 18F-FDG PET\/CT scan in characterizing adrenal tumors","volume":"108","author":"Schaafsma","year":"2023","journal-title":"J Clin Endocrinol Metab"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1530\/EC-19-0204","article-title":"Diagnostic efficacy of 18F-FDG PET\/CT in patients with adrenal incidentaloma","volume":"8","author":"Akku\u015f","year":"2019","journal-title":"Endocrine Connections"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.13.11793","article-title":"18 F-FDG PET\/CT of adrenal lesions","volume":"203","author":"Dong","year":"2014","journal-title":"Am J Roentgenol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1097\/00003072-199902000-00007","article-title":"Recurrent ectopic adrenocorticotropic hormone producing thymic carcinoid detected with octreotide imaging","volume":"24","author":"Silva","year":"1999","journal-title":"Clin Nucl Med"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(99)00052-5","article-title":"Corticosteroid-induced glaucoma attributable to an adrenocorticotropin-secreting Malignant carcinoid tumor of the thymus","volume":"128","author":"Blumenthal","year":"1999","journal-title":"Am J Ophthalmol"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.4_MeetingAbstracts.324S-c","article-title":"REDUCED LUNG VOLUMES IN A RARE CASE OF CUSHING SYNDROME DUE TO THYMIC CARCINOID OF THE LUNG","volume":"130","author":"Sankri-Tarbichi","year":"2006","journal-title":"Chest"},{"key":"B24","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1258\/ar.2012.120536","article-title":"Evaluation of thymic tumors with 18F-FDG PET-CT: A pictorial review","volume":"54","author":"Sharma","year":"2013","journal-title":"Acta Radiol"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.108.058586","article-title":"Physiologic thymic uptake of 18 F-FDG in children and young adults: A PET\/CT evaluation of incidence, patterns, and relationship to treatment","volume":"50","author":"Jerushalmi","year":"2009","journal-title":"J Nucl Med"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13236091","article-title":"Current roles of PET\/CT in thymic epithelial tumours: which evidences and which prospects? A pictorial review","volume":"13","author":"Lococo","year":"2021","journal-title":"Cancers"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.EJ14-0455","article-title":"Ectopic ACTH syndrome caused by desmopressin-responsive thymic neuroendocrine tumor","volume":"62","author":"Sekiguchi","year":"2015","journal-title":"Endocr J"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.14744\/nci.2018.53244","article-title":"Ectopic cushing\u2019s syndrome due to ACTH secreting atypic thymic carcinoid tumor","volume":"6","author":"Duran","year":"2018","journal-title":"North Clin Istanbul"},{"key":"B29","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/s40792-018-0459-7","article-title":"Cushing\u2019s syndrome caused by ACTH-producing thymic typical carcinoid with local invasion and regional lymph node metastasis: a case report","volume":"4","author":"Fujiwara","year":"2018","journal-title":"Surg Case Rep"},{"key":"B30","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1530\/EDM-21-0207","article-title":"Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)","volume":"2022","author":"Li","year":"2022","journal-title":"Endocrinol Diabetes Metab Case Rep"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1007\/s12020-009-9247-2","article-title":"The role of integrated 18F-FDG PET\/CT in identification of ectopic ACTH secretion tumors","volume":"36","author":"Xu","year":"2009","journal-title":"Endocr"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1111\/den.13752","article-title":"Endoscopic ultrasound ablation in a patient with multiple metastatic pancreatic tumors from adrenocorticotropic hormone-producing thymic neuroendocrine neoplasm","volume":"33","author":"Chang","year":"2021","journal-title":"Digestive Endoscopy"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.3390\/medicina55120759","article-title":"ACTH-dependent hypercortisolemia in a patient with a pituitary microadenoma and an atypical carcinoid tumour of the thymus","volume":"55","author":"Baranowska-Jurkun","year":"2019","journal-title":"Medicina"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1530\/EDM-19-0002","article-title":"A unique case of ectopic Cushing\u2019s syndrome from a thymic neuroendocrine carcinoma","volume":"2019","author":"Lawrence","year":"2019","journal-title":"Endocrinol Diabetes Metab Case Rep"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1016\/j.endien.2018.05.006","article-title":"Ectopic Cushing syndrome: Report of 9 cases","volume":"65","author":"Araujo Castro","year":"2018","journal-title":"Endocrinolog\u00eda Diabetes y Nutrici\u00f3n (English ed)"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMcpc1802825","article-title":"Case 18-2018: A 45-year-old woman with hypertension, fatigue, and altered mental status","volume":"378","author":"Pallais","year":"2018","journal-title":"N Engl J Med"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.14310\/horm.2002.1695","article-title":"Urinary steroid metabolites in a case of florid Ectopic Cushing\u2019s syndrome and clinical correlations","volume":"15","author":"Kyriacou","year":"2016","journal-title":"Hormones"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1210\/jcemcr\/luac008","article-title":"Long-term treatment with metyrapone in a man with ectopic cushing syndrome","volume":"1","author":"Wong","year":"2022","journal-title":"JCEM Case Rep"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1007\/s11154-010-9139-z","article-title":"The ectopic ACTH syndrome","volume":"11","author":"Alexandraki","year":"2010","journal-title":"Rev Endocr Metab Disord"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.EJ18-0563","article-title":"Molecular analysis and literature-based hypothesis of an immunonegative prostate small cell carcinoma causing ectopic ACTH syndrome","volume":"66","author":"Takeuchi","year":"2019","journal-title":"Endocr J"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2012.12.001","article-title":"Treatment, prognostic markers and survival in thymic neuroendocrine tumours. A study from a single tertiary referral centre","volume":"79","author":"Crona","year":"2013","journal-title":"Lung Cancer"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.21037\/jtd.2017.08.18","article-title":"Neuroendocrine tumors of the thymus: the oncologist point of view","volume":"9","author":"Girard","year":"2017","journal-title":"J Thorac Dis"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.3978\/j.issn.2072-1439.2014.04.19","article-title":"Management of thymic tumors: a European perspective","volume":"6","author":"Ruffini","year":"2014","journal-title":"J Thorac Dis"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.21037\/med-21-11","article-title":"Systemic treatments for thymic tumors: a narrative review","volume":"5","author":"Zucali","year":"2021","journal-title":"Mediastinum"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.1097\/JTO.0b013e31821ea5f7","article-title":"Chemotherapy definitions and policies for thymic Malignancies","volume":"6","author":"Girard","year":"2011","journal-title":"J Thorac Oncol"}],"container-title":["Frontiers in Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2024.1399930\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,19]],"date-time":"2024-07-19T05:50:06Z","timestamp":1721368206000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2024.1399930\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,14]]},"references-count":45,"alternative-id":["10.3389\/fendo.2024.1399930"],"URL":"https:\/\/doi.org\/10.3389\/fendo.2024.1399930","relation":{},"ISSN":["1664-2392"],"issn-type":[{"value":"1664-2392","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,14]]},"article-number":"1399930"}}