{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T06:16:18Z","timestamp":1769580978177,"version":"3.49.0"},"reference-count":23,"publisher":"Consilium Medicum","issue":"10","license":[{"start":{"date-parts":[[2021,10,15]],"date-time":"2021-10-15T00:00:00Z","timestamp":1634256000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Terapevticheskii arkhiv"],"abstract":"<jats:p>Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome  EAS (ACTH  adrenocorticotropic hormone ).\r\nMaterials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed.\r\nResults. The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9  thymic carcinoid, 7  pancreatic NET, 5  pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1  medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients  in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%)  had relapse of the disease and 26 (20%)  died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1).\r\nConclusion. In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.<\/jats:p>","DOI":"10.26442\/00403660.2021.10.201102","type":"journal-article","created":{"date-parts":[[2021,11,5]],"date-time":"2021-11-05T18:35:47Z","timestamp":1636137347000},"page":"1171-1178","source":"Crossref","is-referenced-by-count":13,"title":["Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization"],"prefix":"10.26442","volume":"93","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2320-1051","authenticated-orcid":false,"given":"Olga O.","family":"Golounina","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6674-6441","authenticated-orcid":false,"given":"Zhanna E.","family":"Belaya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7041-0732","authenticated-orcid":false,"given":"Liudmila Ya.","family":"Rozhinskaya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5130-4157","authenticated-orcid":false,"given":"Evgeniya I.","family":"Marova","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0559-4461","authenticated-orcid":false,"given":"Michail Yu.","family":"Pikunov","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6993-5096","authenticated-orcid":false,"given":"Patimat 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