{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T16:46:48Z","timestamp":1791910008863,"version":"4.3.4"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"3","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:p>Spontaneous remission is rare in ectopic ACTH syndrome (EAS). We describe four patients with presumed EAS in whom long-term treatment with steroidogenesis inhibitors was followed by prolonged remission of hypercortisolemia. Biochemical testing was consistent with EAS, but imaging failed to identify a tumor. Patients were treated with ketoconazole alone or with mitotane and\/or metyrapone to control hypercortisolemia. Dexamethasone was added when a block and replace strategy was used. Treatment with steroidogenesis inhibitors for 3\u201310 years in these patients was followed by a prolonged period of remission (15\u201360 months). During remission, the first patient had an elevated ACTH, low cortisol and 24-h urinary free cortisol (UFC), and adrenal atrophy on computerized tomography scan during remission, suggesting a direct toxic effect on the adrenal glands. Cases 2 and 3 had normal to low ACTH levels and low-normal UFC, consistent with an effect at the level of the ectopic tumor. They did not have a history of cyclicity and case 3 has been in remission for \u223c5 years, making cyclic Cushing's syndrome less likely. Case 4, with a history of cyclic hypercortisolism, had normal to slightly elevated ACTH levels and low-normal UFC during remission. The most likely etiology of remission is cyclic production of ACTH by the ectopic tumor. Spontaneous and sustained remission of hypercortisolemia is possible in EAS after long-term treatment with steroidogenesis inhibitors; a drug holiday may be warranted during chronic therapy to evaluate this. The pathophysiology remains unclear but may involve several different mechanisms.<\/jats:p>","DOI":"10.1530\/eje-11-0949","type":"journal-article","created":{"date-parts":[[2011,12,22]],"date-time":"2011-12-22T05:10:47Z","timestamp":1324530647000},"page":"531-536","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":25,"title":["Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion"],"prefix":"10.1093","volume":"166","author":[{"given":"S T","family":"Sharma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L K","family":"Nieman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","reference":[{"issue":"2","key":"1_10109421","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1001\/archinte.1993.00410020097009","volume":"153","author":"Ishibashi","year":"1993","journal-title":"Archives of Internal Medicine","ISSN":"https:\/\/id.crossref.org\/issn\/0003-9926","issn-type":"print"},{"issue":"1","key":"2_9386602","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1001\/archinte.1991.00400010171028","volume":"151","author":"Dickstein","year":"1991","journal-title":"Archives of Internal Medicine","ISSN":"https:\/\/id.crossref.org\/issn\/0003-9926","issn-type":"print"},{"key":"3_41979611","doi-asserted-by":"crossref","first-page":"147","DOI":"10.4158\/EP.9.2.147","volume":"9","year":"2003","journal-title":"ENDOCRINE PRACTICE"},{"key":"4_41979612","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1023\/B:PITU.0000044626.25624.48","volume":"7","year":"2004","ISSN":"https:\/\/id.crossref.org\/issn\/1573-7403","issn-type":"print"},{"issue":"3","key":"5_29293681","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1530\/EJE-07-0262","volume":"157","author":"Meinardi","year":"2007","journal-title":"European Journal of Endocrinology","ISSN":"https:\/\/id.crossref.org\/issn\/0804-4643","issn-type":"print"},{"issue":"6","key":"6_34299573","doi-asserted-by":"publisher","first-page":"1011","DOI":"10.1530\/EJE-09-0046","volume":"160","year":"2009","journal-title":"European Journal of Endocrinology","ISSN":"https:\/\/id.crossref.org\/issn\/0804-4643","issn-type":"print"},{"issue":"5","key":"7_8533115","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1111\/j.1365-2265.1981.tb00638.x","volume":"14","author":"Beardwell","year":"1981","journal-title":"Clinical endocrinology","ISSN":"https:\/\/id.crossref.org\/issn\/0300-0664","issn-type":"print"},{"issue":"4","key":"8_16518174","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1530\/eje.0.1350440","volume":"135","author":"Loh","year":"1996","journal-title":"European Journal of Endocrinology","ISSN":"https:\/\/id.crossref.org\/issn\/0804-4643","issn-type":"print"},{"issue":"3","key":"9_36405865","doi-asserted-by":"publisher","first-page":"1207","DOI":"10.1210\/jc.2009-2282","volume":"95","year":"2010","journal-title":"Journal of Clinical Endocrinology &amp; 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