{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T06:06:23Z","timestamp":1774418783749,"version":"3.50.1"},"reference-count":28,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2019,11,27]],"date-time":"2019-11-27T00:00:00Z","timestamp":1574812800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Cushing\u2019s syndrome (CS) is a set of clinical symptoms which occur as a result of hypercortisolemia. Endogenous ACTH-dependent CS related to an ectopic ACTH-secreting tumour constitutes 12%\u201317% of CS cases and is one of the most common causes of paraneoplastic syndromes. This study presents a case of a 31 year-old man with diabetes, hypertension, rosacea, purple stretch marks and hypokalemia. Findings of diagnostic procedures include high concentrations of cortisol and ACTH, pituitary microadenoma and a tumour in the anterior mediastinum. Dynamic hormone tests determined the source of excess hormone secretion and ectopic ACTH-dependent CS was diagnosed. Due to increasing symptoms of superior vena cava syndrome, an emergency resection of almost the whole tumour was performed, with only a small part of the upper pole left because of the proximity of large vessels and a risk of damaging them. On the basis of histopathological tests, an atypical carcinoid tumour of the thymus was identified. Immediately after the surgical procedure, there was a significant reduction of clinical and laboratory traits of hypercortisolemia, yet, during the 46 weeks of postoperative observation, despite chemotherapy, the progression of residual masses of the tumour occurred with metastases and increased hormone indices. The presented case shows and discusses the differentiation of ACTH-dependent hypercortisolemia and its causes, difficulties in surgical therapy and chemotherapy, as well as prognosis for atypical carcinoid of the thymus, which is a rare disease.<\/jats:p>","DOI":"10.3390\/medicina55120759","type":"journal-article","created":{"date-parts":[[2019,11,27]],"date-time":"2019-11-27T03:55:51Z","timestamp":1574826951000},"page":"759","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["ACTH-dependent Hypercortisolemia in a Patient with a Pituitary Microadenoma and an Atypical Carcinoid Tumour of the Thymus"],"prefix":"10.3390","volume":"55","author":[{"given":"Angelika","family":"Baranowska-Jurkun","sequence":"first","affiliation":[{"name":"Clinic of Endocrinology, Diabetology and Internal Medicine, Department of Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, \u017bo\u0142nierska 18, 10-561 Olsztyn, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magdalena","family":"Szychli\u0144ska","sequence":"additional","affiliation":[{"name":"Clinic of Endocrinology, Diabetology and Internal Medicine, Department of Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, \u017bo\u0142nierska 18, 10-561 Olsztyn, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wojciech","family":"Matuszewski","sequence":"additional","affiliation":[{"name":"Clinic of Endocrinology, Diabetology and Internal Medicine, Department of Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, \u017bo\u0142nierska 18, 10-561 Olsztyn, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Modzelewski","sequence":"additional","affiliation":[{"name":"Clinic of Endocrinology, Diabetology and Internal Medicine, Department of Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, \u017bo\u0142nierska 18, 10-561 Olsztyn, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"El\u017cbieta","family":"Bandurska-Stankiewicz","sequence":"additional","affiliation":[{"name":"Clinic of Endocrinology, Diabetology and Internal Medicine, Department of Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, \u017bo\u0142nierska 18, 10-561 Olsztyn, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2019,11,27]]},"reference":[{"key":"ref_1","first-page":"117","article-title":"Incidence and Late Prognosis of Cushing\u2019s Syndrome: A Population-Based Study","volume":"86","author":"Lindholm","year":"2001","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1016\/S0140-6736(06)68699-6","article-title":"Cushing\u2019s Syndrome","volume":"367","author":"Bertagna","year":"2006","journal-title":"Lancet Lond. Engl."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"4955","DOI":"10.1210\/jc.2004-2527","article-title":"Cushing\u2019s Syndrome Due to Ectopic Corticotropin Secretion: Twenty Years\u2019 Experience at the National Institutes of Health","volume":"90","author":"Ilias","year":"2005","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1590\/S0004-27302007000800007","article-title":"Ectopic ACTH Syndrome","volume":"51","author":"Isidori","year":"2006","journal-title":"Arq. Bras. Endocrinol. Metabol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"838","DOI":"10.4065\/mcp.2010.0099","article-title":"Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment","volume":"85","author":"Pelosof","year":"2010","journal-title":"Mayo Clin. Proc."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1634\/theoncologist.2018-0291","article-title":"Capecitabine and Temozolomide as a Promising Therapy for Advanced Thymic Atypical Carcinoid","volume":"24","author":"Wang","year":"2019","journal-title":"Oncologist"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1652","DOI":"10.1016\/S1470-2045(17)30681-2","article-title":"Efficacy and Safety of Long-Acting Pasireotide or Everolimus Alone or in Combination in Patients with Advanced Carcinoids of the Lung and Thymus (LUNA): An Open-Label, Multicentre, Randomised, Phase 2 Trial","volume":"18","author":"Ferolla","year":"2017","journal-title":"Lancet Oncol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/s12032-013-0479-x","article-title":"Clinical Outcomes of Atypical Carcinoid Tumors of the Lung and Thymus: 7-Year Experience of a Rare Malignancy at Single Institute","volume":"30","author":"Han","year":"2013","journal-title":"Med. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1002\/gcc.22183","article-title":"Tumor Genetics and Survival of Thymic Neuroendocrine Neoplasms: A Multi-Institutional Clinicopathologic Study","volume":"53","author":"Zettl","year":"2014","journal-title":"Genes. Chromosomes Cancer"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1022","DOI":"10.1016\/S0140-6736(00)84800-X","article-title":"A case of pluriglandular syndrome: \u201cdiabetes of bearded women\u201d","volume":"212","author":"Brown","year":"1928","journal-title":"Lancet"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1001\/archinte.1963.03620280071011","article-title":"Nonpituitary Neoplasms and Cushing\u2019s Syndrome: Ectopic Adrenocorticotropin Produced by Nonpituitary Neoplasms as a Cause of Cushing\u2019s Syndrome","volume":"111","author":"Liddle","year":"1963","journal-title":"Arch. Intern. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/0960-0760(95)00044-Z","article-title":"Ectopic ACTH Syndrome","volume":"53","author":"Wajchenberg","year":"1995","journal-title":"J. Steroid Biochem. Mol. Biol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1016\/j.jtcvs.2003.09.058","article-title":"Multidisciplinary Treatment of Advanced Thymic Neuroendocrine Carcinoma (Carcinoid): Report of a Successful Case and Review of the Literature","volume":"127","author":"Filosso","year":"2004","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1733","DOI":"10.1016\/S0003-4975(02)03547-6","article-title":"Primary Neuroendocrine Tumors of the Thymus","volume":"74","author":"Chaer","year":"2002","journal-title":"Ann. Thorac. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1097\/SLA.0b013e3181dd4ec4","article-title":"Thymic Neuroendocrine Tumors: A SEER Database Analysis of 160 Patients","volume":"251","author":"Gaur","year":"2010","journal-title":"Ann. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1002\/1097-0142(197204)29:4<1061::AID-CNCR2820290456>3.0.CO;2-3","article-title":"Mediastinal Endocrine Neoplasm, of Probable Thymic Origin, Related to Carcinoid Tumor. Clinicopathologic Study of 8 Cases","volume":"29","author":"Rosai","year":"1972","journal-title":"Cancer"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1097\/JTO.0000000000000654","article-title":"The 2015 WHO Classification of Tumors of the Thymus: Continuity and Changes","volume":"10","author":"Marx","year":"2015","journal-title":"J. Thorac. Oncol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"S1484","DOI":"10.21037\/jtd.2017.10.83","article-title":"Neuroendocrine Tumors of the Thymus","volume":"9","author":"Filosso","year":"2017","journal-title":"J. Thorac. Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1080\/14017439950142046","article-title":"Multiple Endocrine Neoplastic-Associated Thymic Carcinoid Tumour in Close Relatives: Octreotide Scan as a New Diagnostic and Follow-up Modality. Two Case Reports","volume":"33","author":"Satta","year":"1999","journal-title":"Scand. Cardiovasc. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1016\/S0003-4975(01)03032-6","article-title":"Neuroendocrine Tumors of the Thymus: A Clinicopathological and Prognostic Study","volume":"72","author":"Gal","year":"2001","journal-title":"Ann. Thorac. Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1097\/01.COC.0000020584.56294.72","article-title":"Atypical Thymic Carcinoid and Malignant Somatostatinoma in Type I Multiple Endocrine Neoplasia Syndrome: Case Report","volume":"26","author":"Lin","year":"2003","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1309\/3PDN-PMT5-EQTM-H0CD","article-title":"Neuroendocrine Carcinomas (Carcinoid Tumor) of the Thymus. A Clinicopathologic Analysis of 80 Cases","volume":"114","author":"Moran","year":"2000","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1016\/j.humpath.2013.01.010","article-title":"Thymomas with Prominent Glandular Differentiation: A Clinicopathologic and Immunohistochemical Study of 12 Cases","volume":"44","author":"Weissferdt","year":"2013","journal-title":"Hum. Pathol."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"\u00d6berg, K., Hellman, P., Ferolla, P., Papotti, M., and ESMO Guidelines Working Group (2012). Neuroendocrine Bronchial and Thymic Tumors: ESMO Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up. Ann. Oncol., vii120\u2013vii123.","DOI":"10.1093\/annonc\/mds267"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1378\/chest.124.1.141","article-title":"A Clinicopathologic Study of 12 Neuroendocrine Tumors Arising in the Thymus","volume":"124","author":"Tiffet","year":"2003","journal-title":"Chest"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1016\/j.athoracsur.2005.08.030","article-title":"Experience with the \u201cDa Vinci\u201d Robotic System for Thymectomy in Patients with Myasthenia Gravis: Report of 33 Cases","volume":"81","author":"Rea","year":"2006","journal-title":"Ann. Thorac. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/j.athoracsur.2012.09.006","article-title":"Thymic Carcinoma: A Multivariate Analysis of Factors Predictive of Survival in 290 Patients","volume":"95","author":"Weksler","year":"2013","journal-title":"Ann. Thorac. Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e13286","DOI":"10.1097\/MD.0000000000013286","article-title":"Metastatic Thymic Carcinoid Responds to Chemoradiation and Octreotide: A Case Report","volume":"97","author":"Mei","year":"2018","journal-title":"Medicine"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/55\/12\/759\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:37:49Z","timestamp":1760189869000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/55\/12\/759"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,27]]},"references-count":28,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2019,12]]}},"alternative-id":["medicina55120759"],"URL":"https:\/\/doi.org\/10.3390\/medicina55120759","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,27]]}}}