{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T18:13:15Z","timestamp":1779905595074,"version":"3.53.1"},"reference-count":39,"publisher":"FapUNIFESP (SciELO)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq Bras Endocrinol Metab"],"published-print":{"date-parts":[[2012,7]]},"abstract":"<jats:p>ACTH-dependent Cushing syndrome (CS) due to ectopic ACTH production is most times difficult to manage. The identification of the source of ACTH may take many years. Surgery or chemotherapy for the primary tumor is not always possible. Control of Cushing symptoms is many times achieved using medication, or bilateral adrenalectomy in refractory cases. This case presents a Brazilian male who showed severe hypertension, mood changes, muscle weakness, darkening of skin, and increased abdominal fat. An investigation for Cushing syndrome was carried out and, after a four-year follow-up, a carotid glomus tumor (chemodectoma) was confirmed, a rare ectopic ACTH-producing tumor. Besides, the patient presented cyclic Cushing syndrome that was exacerbated by diverticulitis episodes. This case presents interesting pitfalls on diagnosis and management of ACTH-dependent CS. This is the only report of a chemodectoma that produced ACTH in the literature.<\/jats:p>","DOI":"10.1590\/s0004-27302012000500008","type":"journal-article","created":{"date-parts":[[2012,8,18]],"date-time":"2012-08-18T07:53:39Z","timestamp":1345276419000},"page":"324-330","source":"Crossref","is-referenced-by-count":11,"title":["A rare case of Cushing syndrome by cyclic ectopic-ACTH"],"prefix":"10.1590","volume":"56","author":[{"given":"Mariana","family":"Farage","sequence":"first","affiliation":[{"name":"Instituto Estadual de Diabetes e Endocrinologia, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario Alberto da Dantas Loures","family":"Costa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Am\u00e9lio Fernando","family":"Godoy-Matos","sequence":"additional","affiliation":[{"name":"Instituto Estadual de Diabetes e Endocrinologia, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"530","reference":[{"issue":"8","key":"ref1","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1590\/S0004-27302007000800007","article-title":"Ectopic ACTH syndrome","volume":"51","author":"Isidori AM","year":"2007","journal-title":"Arq Bras Endocrinol Metabol","ISSN":"https:\/\/id.crossref.org\/issn\/0004-2730","issn-type":"print"},{"issue":"5","key":"ref2","first-page":"647","article-title":"The diagnosis and differential diagnosis of Cushing's syndrome and pseudo-Cushing's states","volume":"19","author":"Newell-Price J","year":"1998","journal-title":"Endocr Rev","ISSN":"https:\/\/id.crossref.org\/issn\/0163-769X","issn-type":"print"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1159\/000094323","article-title":"Ectopic ACTH syndrome","volume":"35","author":"Isidori AM","year":"2006","journal-title":"Front Horm Res","ISSN":"https:\/\/id.crossref.org\/issn\/0301-3073","issn-type":"print"},{"issue":"8","key":"ref4","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1590\/S0004-27302007000800006","article-title":"Pitfalls in the diagnosis of Cushing's syndrome","volume":"51","author":"Vilar L","year":"2007","journal-title":"Arq Bras Endocrinol Metabol","ISSN":"https:\/\/id.crossref.org\/issn\/0004-2730","issn-type":"print"},{"issue":"9522","key":"ref5","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1016\/S0140-6736(06)68699-6","article-title":"Cushing's syndrome","volume":"367","author":"Newell-Price J","year":"2006","journal-title":"Lancet","ISSN":"https:\/\/id.crossref.org\/issn\/0099-5355","issn-type":"print"},{"issue":"5","key":"ref6","doi-asserted-by":"crossref","first-page":"2214","DOI":"10.1210\/jc.2003-031812","article-title":"The role of [(18)F]fluorodeoxyglucose positron emission tomography and [(111)In]-diethylenetriaminepentaacetate-D-Phe-pentetreotide scintigraphy in the localization of ectopic adrenocorticotropin-secreting tumors causing Cushing's syndrome","volume":"89","author":"Pacak K","year":"2004","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1530\/EJE-07-0262","article-title":"Cyclic Cushing's syndrome: a clinical challenge","volume":"157","author":"Meinardi JR","year":"2007","journal-title":"Eur J Endocrinol","ISSN":"https:\/\/id.crossref.org\/issn\/0804-4643","issn-type":"print"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1056\/NEJM199503233321207","article-title":"Cushing's syndrome","volume":"332","author":"Orth DN","year":"1995","journal-title":"N Engl J Med","ISSN":"https:\/\/id.crossref.org\/issn\/0028-4793","issn-type":"print"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"2116","DOI":"10.1002\/1097-0142(19801101)46:9<2116::AID-CNCR2820460934>3.0.CO;2-S","article-title":"Familial carotid body tumors: case report and epidemiologic review","volume":"46","author":"Grufferman S","year":"1980","journal-title":"Cancer","ISSN":"https:\/\/id.crossref.org\/issn\/0008-543X","issn-type":"print"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1177\/019459989010200413","article-title":"Familial multiple cervical paragangliomas: report of a kindred and review of the literature","volume":"102","author":"Sobol SM","year":"1990","journal-title":"Otolaryngol Head Neck Surg","ISSN":"https:\/\/id.crossref.org\/issn\/0194-5998","issn-type":"print"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1822","DOI":"10.1097\/00005537-200110000-00029","article-title":"Proportion of heritable paraganglioma cases and associated clinical characteristics","volume":"111","author":"Drovdlic CM","year":"2001","journal-title":"Laryngoscope","ISSN":"https:\/\/id.crossref.org\/issn\/0023-852X","issn-type":"print"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"2110","DOI":"10.1210\/jc.2004-1398","article-title":"Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma","volume":"90","author":"Amar L","year":"2005","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1097\/00000658-199906000-00001","article-title":"Clinical experience over 48 years with pheochromocytoma","volume":"229","author":"Goldstein RE","year":"1999","journal-title":"Ann Surg","ISSN":"https:\/\/id.crossref.org\/issn\/0003-4932","issn-type":"print"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"2347","DOI":"10.1093\/hmg\/11.20.2347","article-title":"The pressure rises: update on the genetics of phaeochromocytoma","volume":"11","author":"Maher ER","year":"2002","journal-title":"Hum Mol Genet","ISSN":"https:\/\/id.crossref.org\/issn\/0964-6906","issn-type":"print"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1038\/81551","article-title":"Mutations in SDHC cause autosomal dominant paraganglioma, type 3","volume":"26","author":"Niemann S","year":"2000","journal-title":"Nat Genet","ISSN":"https:\/\/id.crossref.org\/issn\/1061-4036","issn-type":"print"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1086\/321282","article-title":"Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma","volume":"69","author":"Astuti D","year":"2001","journal-title":"AM J Hum Genet","ISSN":"https:\/\/id.crossref.org\/issn\/0002-9297","issn-type":"print"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/S0889-8529(18)30086-0","article-title":"Ectopic ACTH syndrome and CRH-mediated Cushing's syndrome","volume":"23","author":"Becker M","year":"1994","journal-title":"Endocrinol Metab Clin North Am","ISSN":"https:\/\/id.crossref.org\/issn\/0889-8529","issn-type":"print"},{"key":"ref18","first-page":"938","article-title":"Cushing syndrome in a patient having had a tumor of the carotid paraganglion","volume":"13","author":"Martin E","year":"1952","journal-title":"Ann Endocrinol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1007\/BF03348527","article-title":"Evidence for ectopic ACTH production years after bilateral adrenalectomy for Cushing's syndrome: in vivo and in vitro studies","volume":"8","author":"Boscaro M","year":"1985","journal-title":"J Endocrinol Invest","ISSN":"https:\/\/id.crossref.org\/issn\/0391-4097","issn-type":"print"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1007\/s002590050267","article-title":"Radionuclide imaging of neuroendocrine tumours: biological basis and diagnostic results","volume":"25","author":"Seregni E","year":"1998","journal-title":"Eur J Nucl Med","ISSN":"https:\/\/id.crossref.org\/issn\/0340-6997","issn-type":"print"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1007\/s00268-001-0032-5","article-title":"Cushing syndrome due to ectopic adrenocorticotropic hormone secretion","volume":"25","author":"Aniszewski JP","year":"2001","journal-title":"World J Surg","ISSN":"https:\/\/id.crossref.org\/issn\/0364-2313","issn-type":"print"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1210\/jc.2005-1542","article-title":"The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up","volume":"91","author":"Isidori AM","year":"2006","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1530\/eje.0.1370688","article-title":"Diagnosis and radioguided surgery with 111In-pentetreotide in a patient with paraneoplastic Cushing's syndrome due to a bronchial carcinoid","volume":"137","author":"Mansi L","year":"1997","journal-title":"Eur J Endocrinol","ISSN":"https:\/\/id.crossref.org\/issn\/0804-4643","issn-type":"print"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/S0026-0495(96)90107-9","article-title":"Somatostatin analogs in ectopic corticotropin production","volume":"45","author":"von Werder K","year":"1996","journal-title":"Metabolism","ISSN":"https:\/\/id.crossref.org\/issn\/0026-0495","issn-type":"print"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"2457","DOI":"10.1056\/NEJM200506093522322","article-title":"Cabergoline plus lanreotide for ectopic Cushing's syndrome","volume":"352","author":"Pivonello R","year":"2005","journal-title":"N Engl J Med","ISSN":"https:\/\/id.crossref.org\/issn\/0028-4793","issn-type":"print"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1200\/JCO.1995.13.1.157","article-title":"Ketoconazole in the management of paraneoplastic Cushing's syndrome secondary to ectopic adrenocorticotropin production","volume":"13","author":"Winquist EW","year":"1995","journal-title":"J Clin Oncol","ISSN":"https:\/\/id.crossref.org\/issn\/0732-183X","issn-type":"print"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1111\/j.1365-2265.1981.tb00638.x","article-title":"Prolonged remission in florid Cushing's syndrome following metyrapone treatment","volume":"14","author":"Beardwell CG","year":"1981","journal-title":"Clin Endocrinol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1111\/j.1365-2265.1987.tb01174.x","article-title":"Response of pituitary-adrenal axis to corticotrophin releasing hormone in patients with Cushing's disease before and after ketoconazole treatment","volume":"27","author":"Boscaro M","year":"1987","journal-title":"Clin Endocrinol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1677\/joe.0.1080037","article-title":"Effect of ketoconazole on adrenocorticotrophic hormone secretion in vitro and in vivo","volume":"108","author":"Burrin JM","year":"1986","journal-title":"J Endocrinol","ISSN":"https:\/\/id.crossref.org\/issn\/0022-0795","issn-type":"print"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1056\/NEJM198709243171307","article-title":"The use of ketoconazole as an inhibitor of steroid production","volume":"317","author":"Sonino N","year":"1987","journal-title":"N Engl J Med","ISSN":"https:\/\/id.crossref.org\/issn\/0028-4793","issn-type":"print"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0889-8529(05)70064-5","article-title":"Medical therapy for Cushing's disease","volume":"28","author":"Sonino N","year":"1999","journal-title":"Endocrinol Metab Clin North Am","ISSN":"https:\/\/id.crossref.org\/issn\/0889-8529","issn-type":"print"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"2454","DOI":"10.1210\/jc.2007-2734","article-title":"Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement","volume":"93","author":"Biller BMK","year":"2008","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1056\/NEJM197903013000903","article-title":"Treatment of Cushing's disease by O,p'DDD. Survey of 62 cases","volume":"300","author":"Luton JP","year":"1979","journal-title":"N Engl J Med","ISSN":"https:\/\/id.crossref.org\/issn\/0028-4793","issn-type":"print"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1345\/aph.1H365","article-title":"Etomidate use for Cushing's syndrome caused by an ectopic adrenocorticotropic hormone-producing tumor","volume":"41","author":"Johnson TN","year":"2007","journal-title":"Ann Pharmacother","ISSN":"https:\/\/id.crossref.org\/issn\/1060-0280","issn-type":"print"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/s11154-008-9082-4","article-title":"Somatostatin and dopamine receptors as targets for medical treatment of Cushing's Syndrome","volume":"10","author":"de Bruin C","year":"2009","journal-title":"Rev Endocr Metab Disord"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"988","DOI":"10.1210\/jcem-68-5-988","article-title":"Suppression of ectopic adrenocorticotropin secretion by the long-acting somatostatin analog octreotide","volume":"68","author":"Bertagna X","year":"1989","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref37","first-page":"2885","article-title":"Additive effect of ketoconazole and octreotide in the treatment of severe adrenocorticotropin-dependent hypercortisolism","volume":"81","author":"Vignati F","year":"1996","journal-title":"J Clin Endocrinol Metab","ISSN":"https:\/\/id.crossref.org\/issn\/0021-972X","issn-type":"print"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/0002-9343(94)90059-0","article-title":"Somatostatin receptor scintigraphy: its value in tumor localization in patients with Cushing's syndrome caused by ectopic corticotropin or corticotropin-releasing hormone secretion","volume":"96","author":"de Herder WW","year":"1994","journal-title":"Am J Med","ISSN":"https:\/\/id.crossref.org\/issn\/0002-9343","issn-type":"print"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1210\/er.2000-0001","article-title":"The pathophysiological consequences of somatostatin receptor internalization and resistance","volume":"24","author":"Hofland LJ","year":"2003","journal-title":"Endocr Rev","ISSN":"https:\/\/id.crossref.org\/issn\/0163-769X","issn-type":"print"}],"container-title":["Arquivos Brasileiros de Endocrinologia &amp; Metabologia"],"original-title":[],"link":[{"URL":"http:\/\/www.scielo.br\/pdf\/abem\/v56n5\/a08v56n5.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,7,2]],"date-time":"2019-07-02T23:30:56Z","timestamp":1562110256000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.scielo.br\/scielo.php?script=sci_arttext&pid=S0004-27302012000500008&lng=en&tlng=en"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,7]]},"references-count":39,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2012,7]]}},"alternative-id":["S0004-27302012000500008"],"URL":"https:\/\/doi.org\/10.1590\/s0004-27302012000500008","relation":{},"ISSN":["0004-2730"],"issn-type":[{"value":"0004-2730","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,7]]}}}