{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T10:35:35Z","timestamp":1772188535263,"version":"3.50.1"},"reference-count":17,"publisher":"The Endocrine Society","issue":"3","license":[{"start":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T00:00:00Z","timestamp":1772150400000},"content-version":"vor","delay-in-days":4,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Ectopic Cushing syndrome is an uncommon cause of hypercortisolism that needs rapid recognition and treatment to reduce complications. Here, we present the case of a 33-year-old man with a 1-year history of severe Cushing syndrome. Biochemical findings showed both high ACTH and 24-hour urine free cortisol, and nonsuppressed morning serum cortisol. The 18fluorine-1,4,7-triazacyclononane-1,4,7-triacetate-octreotide positron emission tomography\/computed tomography revealed a large mediastinal tumor with high uptake. Initial biopsy reported a grade 1 neuroendocrine tumor with positive ACTH immunostaining. Treatment was initiated with ketoconazole and somatostatin analog. To achieve rapid Cushing syndrome control, etomidate intravenous infusion was started. After multidisciplinary assessment and because of high surgical risk (concern for airway compromise from tumor location above the trachea, size, and possible mechanical lung compression with laparoscopic adrenalectomy) debulking surgery of the primary tumor was performed first, followed by bilateral adrenalectomy. Pathology findings of the R2 tumor showed a higher grade tumor than previously reported and an 18 Fluorodeoxyglucose positron emission tomography\/computed tomography demonstrated distant metastatic disease. In summary, severe hypercortisolism usually occurs in the setting of ectopic production, it can be debilitating with increased mortality, and in this case, tumor aggressiveness and location made management particularly challenging, requiring a multidisciplinary approach.<\/jats:p>","DOI":"10.1210\/jcemcr\/luaf283","type":"journal-article","created":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T13:06:37Z","timestamp":1762520797000},"source":"Crossref","is-referenced-by-count":0,"title":["Ectopic Cushing Syndrome Due to a Large Mediastinal Neuroendocrine Tumor"],"prefix":"10.1210","volume":"4","author":[{"given":"Jorge Alberto","family":"Ram\u00edrez-Garc\u00eda","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriela","family":"Garza-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roopa","family":"Mehta","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alfredo A","family":"Reza-Albarr\u00e1n","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"De La Pe\u00f1a-L\u00f3pez","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8614-7163","authenticated-orcid":false,"given":"Nicole M","family":"I\u00f1iguez-Ariza","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n \u201cSalvador Zubir\u00e1n\u201d , Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2026,2,27]]},"reference":[{"issue":"2","key":"2026022704512493100_luaf283-B1","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1210\/jc.2005-1542","article-title":"The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up","volume":"91","author":"Isidori","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2026022704512493100_luaf283-B2","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1001\/jama.2023.11305","article-title":"Cushing syndrome: a review","volume":"330","author":"Reincke","year":"2023","journal-title":"JAMA"},{"issue":"4","key":"2026022704512493100_luaf283-B3","doi-asserted-by":"crossref","first-page":"1041","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"},{"issue":"11","key":"2026022704512493100_luaf283-B4","doi-asserted-by":"crossref","first-page":"3162","DOI":"10.1210\/clinem\/dgac492","article-title":"Approach to the patient: diagnosis of Cushing syndrome","volume":"107","author":"Savas","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2026022704512493100_luaf283-B5","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1007\/s40265-016-0539-6","article-title":"Therapeutic strategies for the treatment of severe Cushing\u2019s syndrome","volume":"76","author":"Alexandraki","year":"2016","journal-title":"Drugs"},{"key":"2026022704512493100_luaf283-B6","doi-asserted-by":"crossref","first-page":"20420188211058583","DOI":"10.1177\/20420188211058583","article-title":"Pharmacological management of severe Cushing\u2019s syndrome: the role of etomidate","volume":"13","author":"Pence","year":"2022","journal-title":"Ther Adv Endocrinol Metab"},{"issue":"3","key":"2026022704512493100_luaf283-B7","doi-asserted-by":"crossref","first-page":"bvac010","DOI":"10.1210\/jendso\/bvac010","article-title":"Perioperative management of a patient with Cushing disease","volume":"6","author":"Varlamov","year":"2022","journal-title":"J Endocr Soc"},{"issue":"4","key":"2026022704512493100_luaf283-B8","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1177\/02676591231160545","article-title":"Is this dyspnea because of coronary artery disease? A rare case report of primary neuroendocrine tumor in anterior mediastinum","volume":"39","author":"Rumbinait\u0117","year":"2024","journal-title":"Perfusion"},{"issue":"4","key":"2026022704512493100_luaf283-B9","doi-asserted-by":"crossref","first-page":"2893","DOI":"10.1378\/chest.128.4.2893","article-title":"Tumors of the mediastinum","volume":"128","author":"Duwe","year":"2005","journal-title":"Chest"},{"issue":"3","key":"2026022704512493100_luaf283-B10","doi-asserted-by":"crossref","first-page":"e12","DOI":"10.1210\/clinem\/dgz136","article-title":"Time to diagnosis in Cushing\u2019s syndrome: a meta-analysis based on 5367 patients","volume":"105","author":"Rubinstein","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2026022704512493100_luaf283-B11","first-page":"1780","article-title":"Effectiveness versus efficacy: the limited value in clinical practice of high dose dexamethasone suppression testing in the differential diagnosis of adrenocorticotropin-dependent Cushing\u2019s syndrome","volume":"82","author":"Aron","year":"1997","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2026022704512493100_luaf283-B12","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1002\/ccr3.1478","article-title":"Etomidate in the control of severe Cushing\u2019s syndrome by neuroendocrine carcinoma","volume":"6","author":"Reza-Albarr\u00e1n","year":"2018","journal-title":"Clin Case Rep"},{"issue":"3","key":"2026022704512493100_luaf283-B13","doi-asserted-by":"crossref","first-page":"E175","DOI":"10.21037\/jtd.2016.02.55","article-title":"Management of large mediastinal masses: surgical and anesthesiological considerations","volume":"8","author":"Li","year":"2016","journal-title":"J Thorac Dis"},{"issue":"1","key":"2026022704512493100_luaf283-B14","first-page":"3","article-title":"Profile and factors associated with mortality in mediastinal mass during hospitalization at Cipto Mangunkusumo Hospital, Jakarta","volume":"45","author":"Singh","year":"2013","journal-title":"Acta Med Indones"},{"issue":"10","key":"2026022704512493100_luaf283-B15","doi-asserted-by":"crossref","first-page":"3939","DOI":"10.1210\/jc.2013-1470","article-title":"Clinical review: outcome of bilateral adrenalectomy in Cushing\u2019s syndrome: a systematic review","volume":"98","author":"Ritzel","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"issue":"Mar","key":"2026022704512493100_luaf283-B16","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1159\/000544982","article-title":"A review for the clinician: classifications, genetics and treatment for neuroendocrine neoplasms of the Thymus (thymic carcinoid)","volume":"11","author":"Lang","year":"2025","journal-title":"Neuroendocrinology"},{"issue":"15_suppl","key":"2026022704512493100_luaf283-B17","doi-asserted-by":"crossref","first-page":"4112","DOI":"10.1200\/JCO.2021.39.15_suppl.4112","article-title":"Final overall survival in the phase 3 NETTER-1 study of lutetium-177-DOTATATE in patients with midgut neuroendocrine tumors","volume":"39","author":"Strosberg","year":"2021","journal-title":"J Clin Oncol"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/4\/3\/luaf283\/67153029\/luaf283.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/4\/3\/luaf283\/67153029\/luaf283.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T09:51:33Z","timestamp":1772185893000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luaf283\/8500931"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,23]]},"references-count":17,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,2,23]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luaf283","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,3]]},"published":{"date-parts":[[2026,2,23]]},"article-number":"luaf283"}}