{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:02Z","timestamp":1784557022938,"version":"3.55.0"},"reference-count":20,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2019,2,22]],"date-time":"2019-02-22T00:00:00Z","timestamp":1550793600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0\/deed.en_GB"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,2,22]]},"abstract":"Summary<\/jats:title>\n Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing\u2019s syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS. As the diagnosis of CS from ectopic ACTH syndrome (EAS) remains challenging, we emphasize the necessity for high clinical suspicion in the appropriate setting, concordance between biochemical, imaging and pathology findings, along with continued vigilant monitoring for recurrence after definitive treatment.<\/jats:p>\n \n Learning points:<\/jats:title>\n \n \n Functional thymic neuroendocrine tumors are exceedingly rare.<\/jats:p>\n <\/jats:list-item>\n \n Ectopic Cushing\u2019s syndrome secondary to thymic neuroendocrine tumors secreting ACTH present with features of hypercortisolism including electrolyte and metabolic abnormalities, uncontrolled hypertension and hyperglycemia, and opportunistic infections.<\/jats:p>\n <\/jats:list-item>\n \n The ability to undergo surgery and completeness of resection are the strongest prognostic factors for improved overall survival; however, the recurrence rate remains high.<\/jats:p>\n <\/jats:list-item>\n \n A high degree of initial clinical suspicion followed by vigilant monitoring is required for patients with this challenging disease.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-19-0002","type":"journal-article","created":{"date-parts":[[2019,2,25]],"date-time":"2019-02-25T06:56:42Z","timestamp":1551077802000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":8,"title":["A unique case of ectopic Cushing\u2019s syndrome from a thymic neuroendocrine carcinoma"],"prefix":"10.1530","volume":"2019","author":[{"given":"Lima","family":"Lawrence","sequence":"first","affiliation":[{"name":"1Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peng","family":"Zhang","sequence":"additional","affiliation":[{"name":"2Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Humberto","family":"Choi","sequence":"additional","affiliation":[{"name":"2Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Usman","family":"Ahmad","sequence":"additional","affiliation":[{"name":"3Departments of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Arrossi","sequence":"additional","affiliation":[{"name":"4Departments of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrei","family":"Purysko","sequence":"additional","affiliation":[{"name":"5Departments of Diagnostic Radiology, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vinni","family":"Makin","sequence":"additional","affiliation":[{"name":"1Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"ref91","doi-asserted-by":"crossref","first-page":"313","DOI":"10.4158\/EP12149.RA","article-title":"A new therapeutic approach in the medical treatment of Cushing\u2019s syndrome: glucocorticoid receptor blockade with mifepristone","volume":"19","year":"2013","journal-title":"Endocrine Practice"},{"key":"ref181","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.4158\/EP15662.OR","article-title":"Pituitary MRI findings in patients with pituitary and ectopic ACTH-dependent Cushing syndrome: does a 6-mm pituitary tumor size cut-off value exclude ectopic ACTH syndrome?","volume":"21","year":"2015","journal-title":"Endocrine Practice"},{"key":"ref101","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1016\/S0140-6736(14)61375-1","article-title":"Cushing\u2019s syndrome","volume":"386","year":"2015","journal-title":"Lancet"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"2223","DOI":"10.1210\/jc.2011-3355","article-title":"Neuroendocrine ACTH-producing tumor of the thymus \u2013 experience with 12 patients over 25 years","volume":"97","year":"2012","journal-title":"Journal of Clinical Endocrinology and Metabolism"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1001\/archinte.1963.03620280071011","article-title":"Nonpituitary neoplasms and Cushing\u2019s syndrome. 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