{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T09:23:39Z","timestamp":1788341019627,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T00:00:00Z","timestamp":1788307200000},"content-version":"vor","delay-in-days":244,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Endocrinology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:p>Ectopic Cushing\u2019s syndrome (ECS) is a rare but important cause of ACTH\u2010dependent Cushing\u2019s syndrome. Metyrapone, a steroidogenesis inhibitor targeting 11\u2010\u03b2 hydroxylase, has shown effectiveness in controlling hypercortisolism. However, little is known about the practical aspects of its initiation, use, and effectiveness in ECS. We present three cases of severe hypercortisolism secondary to ECS managed with metyrapone and report our experience. Case 1: a 56\u2010year\u2010old male with high\u2010grade neuroendocrine carcinoma. Case 2: a 67\u2010year\u2010old man with a history of stage IIB non\u2010small cell lung cancer (NSCLC) with metastatic neuroendocrine carcinoma. Case 3: a 64\u2010year\u2010old man with metastatic prostate cancer. Biochemical control was achieved with rapid titration of low doses of daily metyrapone in all three cases. Based on our experience, we recommend an initial dose of 1000\u20131500\u2009mg of metyrapone daily, in divided doses, with physiologic glucocorticoid replacement to mitigate the risk of adrenal insufficiency. Despite improvements in cortisol levels and stabilization of metabolic derangements, all three patients ultimately transitioned to comfort\u2010focused care. Given the rarity, heterogeneity, and aggressiveness of this condition, rapid diagnosis and standardized pharmacologic approach are difficult to achieve. More data are needed to guide optimal therapy selection, dosing, and titration in ECS.<\/jats:p>","DOI":"10.1155\/crie\/1796526","type":"journal-article","created":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T09:07:22Z","timestamp":1788340042000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Metyrapone in the Management of Severe Hypercortisolism Secondary to Ectopic Cushing\u2019s Syndrome"],"prefix":"10.1155","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9336-3350","authenticated-orcid":false,"given":"Guy I.","family":"Sydney","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-2486-4969","authenticated-orcid":false,"given":"Spyridon","family":"Ntelis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0997-3098","authenticated-orcid":false,"given":"Sachin K.","family":"Majumdar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,2]]},"reference":[{"key":"e_1_2_13_1_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2023.11305"},{"key":"e_1_2_13_2_2","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-19-0877"},{"key":"e_1_2_13_3_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2015-2616"},{"key":"e_1_2_13_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/ejendo\/lvaf181"},{"key":"e_1_2_13_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11154-025-09989-y"},{"key":"e_1_2_13_6_2","doi-asserted-by":"publisher","DOI":"10.1210\/jendso\/bvae194"},{"key":"e_1_2_13_7_2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2022.903545"},{"key":"e_1_2_13_8_2","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgaf633"},{"key":"e_1_2_13_9_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2015-1818"},{"key":"e_1_2_13_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/ejendo\/lvaf114"},{"key":"e_1_2_13_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/jne.13030"},{"key":"e_1_2_13_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjsurg.2020.08.047"},{"key":"e_1_2_13_13_2","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-16-0809"}],"container-title":["Case Reports in Endocrinology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crie\/1796526","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/crie\/1796526","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crie\/1796526","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T09:07:23Z","timestamp":1788340043000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/crie\/1796526"}},"subtitle":[],"editor":[{"given":"Kalyani","family":"Sen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1155\/crie\/1796526"],"URL":"https:\/\/doi.org\/10.1155\/crie\/1796526","archive":["Portico"],"relation":{},"ISSN":["2090-6501","2090-651X"],"issn-type":[{"value":"2090-6501","type":"print"},{"value":"2090-651X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2026-02-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-12","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"1796526"}}