{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T17:57:41Z","timestamp":1725213461473},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T00:00:00Z","timestamp":1609977600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T00:00:00Z","timestamp":1609977600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Endocr Disord"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Neuroendocrine tumor (NET) with adrenocorticotropic hormone (ACTH) secretion are very rare. To our knowledge, no follow-up study is published for ACTH-secreting NET, regardless of the primary site, to show second occurrence of tumor after a long follow-up, following resection of primary tumor.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>Here, we describe a 49-year-old-man with cushingoid feature, drowsiness and quadriparesis came to emergency department at December 2005. Laboratory tests revealed hyperglycemia, metabolic alkalosis, severe hypokalemia, and chemical evidence of an ACTH-dependent hypercortisolism as morning serum cortisol of 57\u2009\u03bcg \/dL without suppression after 8\u2009mg dexamethasone suppression test, serum ACTH level of 256\u2009pg\/mL, and urine free cortisol of &gt;\u20091000\u2009\u03bcg \/24\u2009h. Imaging showed only bilateral adrenal hyperplasia, without evidence of pituitary adenoma or ectopic ACTH producing tumors. Importantly, other diagnostic tests for differentiating Cushing disease (CD) from ectopic ACTH producing tumor, such as inferior petrosal sinus sampling (IPSS), corticotropin releasing hormone (CRH) stimulation test, octreotide scan or fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan were not available in our country at that time. Therefore, bilateral adrenalectomy was performed that led to clinical and biochemical remission of hypercortisolism and decreased ACTH level to &lt;\u200950\u2009pg\/mL, findings suggestive of a primary focus of NET in adrenal glands. After 11\u2009years uncomplicated follow up, the ACTH level elevated up to 341\u2009pg\/mL and re-evaluation showed a 2\u2009cm nodule in the middle lobe of the right lung. Surgical excision of the pulmonary nodule yielded a carcinoid tumor with positive immunostaining for ACTH; leading to decrease in serum ACTH level to 98\u2009pg\/mL. Subsequently after 7\u2009months, serum ACHT levels rose again. More investigation showed multiple lung nodules with metastatic bone lesions accompanied by high serum chromogranin level (2062\u2009ng\/mL), and the patient managed as a metastatic NET, with bisphosphonate and somatostatin receptor analogues.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>This case of surgically-treated NET showing a secondary focus of carcinoid tumor after one decade of disease-free follow-up emphasizes on the importance of long-term follow-up of ACTH-secreting adrenal NET.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12902-020-00673-7","type":"journal-article","created":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T19:03:18Z","timestamp":1610046198000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Recurrence of a neuroendocrine tumor of adrenal origin: a case report with more than a decade follow-up"],"prefix":"10.1186","volume":"21","author":[{"given":"Fatemeh","family":"Rahmani","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maryam","family":"Tohidi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maryam","family":"Dehghani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Behrooz","family":"Broumand","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Farzad","family":"Hadaegh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,1,7]]},"reference":[{"issue":"12","key":"673_CR1","doi-asserted-by":"publisher","first-page":"991","DOI":"10.1016\/j.neo.2017.09.002","volume":"19","author":"B Oronsky","year":"2017","unstructured":"Oronsky B, et al. Nothing but NET: a review of neuroendocrine tumors and carcinomas. Neoplasia. 2017;19(12):991\u20131002.","journal-title":"Neoplasia"},{"issue":"2","key":"673_CR2","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1007\/s11154-010-9139-z","volume":"11","author":"KI Alexandraki","year":"2010","unstructured":"Alexandraki KI, Grossman AB. The ectopic ACTH syndrome. Rev Endocr Metab Disord. 2010;11(2):117\u201326.","journal-title":"Rev Endocr Metab Disord"},{"issue":"3","key":"673_CR3","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1530\/EJE-15-0968","volume":"174","author":"K Kamp","year":"2016","unstructured":"Kamp K, et al. Prevalence and clinical features of the ectopic ACTH syndrome in patients with gastroenteropancreatic and thoracic neuroendocrine tumors. Eur J Endocrinol. 2016;174(3):271\u201380.","journal-title":"Eur J Endocrinol"},{"issue":"2","key":"673_CR4","doi-asserted-by":"publisher","first-page":"76","DOI":"10.22546\/44\/1042","volume":"78","author":"P Oliveira","year":"2017","unstructured":"Oliveira P, Ferreira P, Rocha G. Neuroendocrine Tumor, diagnostic difficulties. Galicia Cl\u00ednica. 2017;78(2):76\u20138.","journal-title":"Galicia Cl\u00ednica"},{"issue":"6","key":"673_CR5","doi-asserted-by":"publisher","first-page":"312","DOI":"10.5732\/cjc.012.10295","volume":"32","author":"C Ro","year":"2013","unstructured":"Ro C, et al. Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment. Chin J Cancer. 2013;32(6):312.","journal-title":"Chin J Cancer"},{"key":"673_CR6","doi-asserted-by":"crossref","unstructured":"Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc. 2010;85(9):838-54.","DOI":"10.4065\/mcp.2010.0099"},{"key":"673_CR7","doi-asserted-by":"crossref","unstructured":"Fazel P, Ganesa P, Mennel RG, Austin NA. The ectopic adrenocorticotropic hormone syndrome in carcinoid tumors. Proc (Bayl Univ Med Cent). 2008;21(2):140-3.","DOI":"10.1080\/08998280.2008.11928380"},{"issue":"7","key":"673_CR8","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1210\/js.2018-00086","volume":"2","author":"JN Gabi","year":"2018","unstructured":"Gabi JN, et al. Severe Cushing syndrome due to an ACTH-producing pheochromocytoma: a case presentation and review of the literature. J Endocr Soc. 2018;2(7):621\u201330.","journal-title":"J Endocr Soc"},{"issue":"12","key":"673_CR9","doi-asserted-by":"publisher","first-page":"1770","DOI":"10.1038\/s41379-018-0110-y","volume":"31","author":"G Rindi","year":"2018","unstructured":"Rindi G, et al. A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Mod Pathol. 2018;31(12):1770\u201386.","journal-title":"Mod Pathol"},{"issue":"6","key":"673_CR10","first-page":"451","volume":"17","author":"S Borzouei","year":"2014","unstructured":"Borzouei S, et al. Multiple endocrine neoplasia type IIa associated with Cushing's syndrome. Arch Iranian Med. 2014;17(6):451\u20134.","journal-title":"Arch Iranian Med"},{"issue":"1","key":"673_CR11","doi-asserted-by":"publisher","first-page":"296","DOI":"10.1007\/s12020-016-1127-y","volume":"55","author":"H Falhammar","year":"2017","unstructured":"Falhammar H, Calissendorff J, H\u00f6ybye C. Frequency of Cushing\u2019s syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center. Endocrine. 2017;55(1):296\u2013302.","journal-title":"Endocrine"},{"issue":"2","key":"673_CR12","doi-asserted-by":"publisher","first-page":"182","DOI":"10.5858\/arpa.2012-0551-OA","volume":"138","author":"O Mete","year":"2014","unstructured":"Mete O, et al. Protocol for the examination of specimens from patients with pheochromocytomas and extra-adrenal paragangliomas. Arch Pathol Lab Med. 2014;138(2):182\u20138.","journal-title":"Arch Pathol Lab Med"},{"key":"673_CR13","volume-title":"Annales de pathologie","author":"P Gosset","year":"1999","unstructured":"Gosset P, et al. 112 cases of sporadic and genetically determined pheochromocytoma: a comparative pathologic study. In: Annales de pathologie; 1999."},{"key":"673_CR14","first-page":"1","volume":"15","author":"F Rahmani","year":"2020","unstructured":"Rahmani F, Mahdavi M, Edraki K, Valizadeh M. A case series of bilateral inferior petrosal sinus sampling with desmopressin in evaluation of ACTH-dependent Cushing\u2019s syndrome in Iran. Hormones. 2020;15:1\u20136.","journal-title":"Hormones"},{"key":"673_CR15","first-page":"1","volume":"1","author":"H Akbari","year":"2020","unstructured":"Akbari H, Ghorbani M, Kabootari M, Mehrjardi AZ, Mohajeri Tehrani MR, Malek M, Khamseh ME. Usefulness of prolactin measurement in inferior petrosal sinus sampling with desmopressin for Cushing\u2019s syndrome. 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