{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:54Z","timestamp":1784557074529,"version":"3.55.0"},"reference-count":13,"publisher":"Bioscientifica","issue":"4","license":[{"start":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T00:00:00Z","timestamp":1696118400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,10,1]]},"abstract":"\n Summary<\/jats:title>\n Severe Cushing\u2019s syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare but often demands rapid diagnostics and treatment of hypercortisolism with its comorbidities. Pharmacotherapy of hypercortisolism by ketoconazole, metyrapone and osilodrostat is currently available. If unsuccessful or insufficient a bilateral adrenalectomy is an option. We present a 28-year-old female with severe Cushing\u2019s syndrome caused by a bronchial metastatic neuroendocrine tumour (NET). Hypercortisolism was efficiently treated by osilodrostat with block\u2013replace and then titration regimen. A once-daily dose was finally used with normalised cortisol levels. Androgen levels measured by liquid chromatography\u2013mass spectrometry were slightly elevated during the treatment but without any symptoms. A simple once-daily use of osilodrostat with titration regimen led to normalised cortisol levels in a severe Cushing\u2019s syndrome patient with an uncurable bronchial NET. Transient hypocortisolism during treatment appeared but was easily treated by hydrocortisone.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Cushing\u2019s syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare.<\/jats:p>\n <\/jats:list-item>\n \n Cortisol upregulation is often severe and rapid, though clinical signs are not always fully pronounced.<\/jats:p>\n <\/jats:list-item>\n \n Rapid treatment is a key for preventing and reducing complications such as fractures, thromboembolism, bleeding, hyperglycaemia, and arterial hypertension.<\/jats:p>\n <\/jats:list-item>\n \n The novel potent steroidogenesis inhibitor osilodrostat can be used as first-line treatment for reducing hypercortisolism.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-23-0076","type":"journal-article","created":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T05:31:07Z","timestamp":1697088667000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":3,"title":["Severe Cushing\u2019s syndrome from an ectopic adrenocorticotropic hormone-secreting neuroendocrine tumour treated by osilodrostat"],"prefix":"10.1530","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0001-9935","authenticated-orcid":true,"given":"V\u00e1clav","family":"H\u00e1na","sequence":"first","affiliation":[{"name":"3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom\u00e1\u0161","family":"Brutvan","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ad\u00e9la","family":"Krausov\u00e1","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Kr\u0161ek","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e1clav","family":"H\u00e1na","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"European Journal of Endocrinology","first-page":"M33","article-title":"Cushing\u2019s syndrome: update on signs, symptoms and biochemical screening","volume":"173","author":"Nieman","year":"2015","unstructured":"Nieman LK. Cushing\u2019s syndrome: update on signs, symptoms and biochemical screening. European Journal of Endocrinology2015173M33\u2013M38. (https:\/\/doi.org\/10.1530\/EJE-15-0464)"},{"key":"bib2","series-title":"Lancet","first-page":"913","article-title":"Cushing\u2019s syndrome","volume":"386","author":"Lacroix","year":"2015","unstructured":"Lacroix AFeelders RAStratakis CA & Nieman LK. Cushing\u2019s syndrome. Lancet2015386913\u2013927. (https:\/\/doi.org\/10.1016\/S0140-6736(1461375-1)"},{"key":"bib3","series-title":"Molecules","first-page":"1379","article-title":"A new heart-cutting method for a multiplex quantitative analysis of steroid hormones in plasma using 2D-LC\/MS\/MS technique","volume":"28","author":"Kotasova","year":"2023","unstructured":"Kotasova MLacina OSpringer DSevcik JBrutvan TJezkova J & Zima T. A new heart-cutting method for a multiplex quantitative analysis of steroid hormones in plasma using 2D-LC\/MS\/MS technique. Molecules2023281379. 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Diabetes and Endocrinology202210384\u2013385. (https:\/\/doi.org\/10.1016\/S2213-8587(2200134-6)"},{"key":"bib12","series-title":"European Journal of Endocrinology","first-page":"315","article-title":"Differences in the spectrum of steroidogenic enzyme inhibition between osilodrostat and metyrapone in ACTH-dependent Cushing syndrome patients","volume":"187","author":"Bonnet-Serrano","year":"2022","unstructured":"Bonnet-Serrano FPoirier JVaczlavik ALaguillier-Morizot CBlanchet BBaron SGuignat LBessiene LBricaire LGroussin L, Differences in the spectrum of steroidogenic enzyme inhibition between osilodrostat and metyrapone in ACTH-dependent Cushing syndrome patients. European Journal of Endocrinology2022187315\u2013322. 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