{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T18:04:13Z","timestamp":1790273053067,"version":"4.1.0"},"reference-count":33,"publisher":"The Endocrine Society","license":[{"start":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T00:00:00Z","timestamp":1788998400000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Spanish Society of Endocrinology & Nutrition"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To analyze the association between the degree of hypercortisolism and survival outcomes of patients with Cushing syndrome (CS) due to adrenocortical carcinoma (ACC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An international retrospective of patients with CS due to ACC. The primary outcomes were to evaluate the prognostic role of baseline hypercortisolism on survival outcomes, including progression-free survival, disease-free survival, and overall survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 101 patients with ACC were included. The median urinary-free cortisol (UFC) levels at the time of CS diagnosis were 6.4 times above the upper limit of normal (ULN; IQR 2.9-14) and 53.5% (n = 54) of the cases had metastatic ACC. Maximum adrenal tumor size (odds ratio [OR] 1.02, 95% CI 1.01-1.03) and UFC levels (OR 1.62, 95% CI 1.03-2.57) were independent predictors of metastatic ACC. The median overall survival was 19.4 (range 1-176) months, the median disease-free survival was 12.3 (range 2.6-143) months, and progression-free survival was 5.6 (range 1.1-43.7) months. The risk of recurrence\/progression increased as the severity of CS increased (21% in mild hypercortisolism [UFC &amp;lt;2\u00d7ULN], 52% in moderate [2-5\u00d7ULN], and 57% in severe CS [&amp;gt;5\u00d7ULN], P = .035). UFC levels (hazard ratio 1.48; 95% CI 0.97-2.24, P = .057) tended to be associated with the risk of progression (metastatic ACC). When we considered a combined outcome (progression in metastatic ACC and recurrence in localized ACC), age, UFC, hypokalemia, R0 after surgery, and Ki-67 were independent predictors of recurrence\/progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Adrenal tumor size and the severity of cortisol excess are associated with a higher probability of metastatic disease at diagnosis. Our study suggests that the severity of cortisol excess appears to be associated with an increased risk of tumor recurrence\/progression, suggesting that hypercortisolism intensity may serve as a marker of aggressive disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgag373","type":"journal-article","created":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T04:02:46Z","timestamp":1789012966000},"source":"Crossref","is-referenced-by-count":0,"title":["Severity and management of hypercortisolism in patients with adrenocortical carcinoma and overt Cushing syndrome"],"prefix":"10.1210","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0519-0072","authenticated-orcid":false,"given":"Marta","family":"Araujo-Castro","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Nutrition, Hospital Universitario Ram\u00f3n y Cajal , Madrid 28034 ,","place":["Spain"]},{"name":"Instituto de Investigaci\u00f3n Biom\u00e9dica Ram\u00f3n y Cajal (IRYCIS) , Madrid 28034 ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9332-2524","authenticated-orcid":false,"given":"Irina","family":"Bancos","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic , Rochester, MN 55905 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1490-5575","authenticated-orcid":false,"given":"Mario","family":"Detomas","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg Department of Internal Medicine I, , W\u00fcrzburg 97080 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9817-9875","authenticated-orcid":false,"given":"Martin","family":"Reincke","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, LMU University Hospital, LMU Munich , Munich 80336 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3034-5598","authenticated-orcid":false,"given":"Darko","family":"Kastelan","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes, University Hospital Zagreb , Zagreb 10000 ,","place":["Croatia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melani","family":"Naglic","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes, University Hospital Zagreb , Zagreb 10000 ,","place":["Croatia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Barac Nekic","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes, University Hospital Zagreb , Zagreb 10000 ,","place":["Croatia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8837-4343","authenticated-orcid":false,"given":"Betina","family":"Biagetti","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Nutrition, Hospital Universitario Vall d\u2019Hebron , Barcelona 08035 ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4719-065X","authenticated-orcid":false,"given":"Anna","family":"Casteras Roman","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Nutrition, Hospital Universitario Vall d\u2019Hebron , Barcelona 08035 ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9s","family":"Cardona","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Nutrition, Hospital Universitario Vall d\u2019Hebron , Barcelona 08035 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