{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T03:36:59Z","timestamp":1793504219789,"version":"4.5.0"},"reference-count":10,"publisher":"Universa BV","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["AGEB"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:p>Cushing\u2019s syndrome (CS) secondary to adrenocorticotropic hormone (ACTH) producing tumours is a severe condition with a challenging diagnosis. Ectopic ACTH-secretion often involves neuroendocrine tumours (NET) in the respiratory tract. ACTH-secreting small intestine neuro-endocrine tumours (siNET) are extremely rare entities barely reported in literature. This review is illustrated by the case of a 75-year old woman with fulminant ectopic CS caused by a ACTH-secreting metastatic siNET. Severe hypokalemia, fluid retention and refractory hypertension were the presenting symptoms. Basal and dynamic laboratory studies were diagnostic for ACTH-dependent CS. Extensive imaging studies of the pituitary and thorax-abdomen areas were normal, while [68Ga]Ga-DOTATATE PET-CT revealed increased small intestine uptake in the left iliac fossa. The hypercortisolism was well controlled with somatostatin analogues, after which a debulking resection of the tumour was performed. Pathological investigation confirmed a well-differentiated NET with sporadic ACTH immunostaining and post-operative treatment with somatostatin analogues was continued with favourable disease control.<\/jats:p>","DOI":"10.51821\/87.1.11872","type":"journal-article","created":{"date-parts":[[2024,4,9]],"date-time":"2024-04-09T20:01:10Z","timestamp":1712692870000},"page":"48-51","source":"Crossref","is-referenced-by-count":1,"title":["Fulminant ectopic Cushing\u2019s syndrome caused by metastatic small intestine neuroendocrine tumour \u2013 a case report and review of the literature"],"prefix":"10.51821","volume":"87","author":[{"given":"B","family":"Alliet","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Severi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Veekmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Cuypers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Topal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.M.","family":"Deroose","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Roskams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Bex","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Dekervel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27062","published-online":{"date-parts":[[2024,4]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","unstructured":"YOUNG J., HAISSAGUERRE M., VIERA-PINTO O., CHABRE O., BAUDIN E., TABARIN A. Management of endocrine disease: Cushing\u2019s syndrome due to ectopic ACTH secretions: an expert operational opinion. European Journal of Endocrinology, 2020, 182(4): 29-58.","DOI":"10.1530\/eje-19-0877"},{"key":"ref1","doi-asserted-by":"publisher","unstructured":"HAYES A.R., GROSSMAN A.B. The Ectopic Adrenocorticotropic Hormone Syndrome: Rarely Easy, Always Challenging. Endocrinol. Metab. Clin. North Am. 2018, 47(2): 409-425.","DOI":"10.1016\/j.ecl.2018.01.005"},{"key":"ref2","doi-asserted-by":"publisher","unstructured":"GRIGORYAN S., AVRAM A.M., TURCU A.F. Functional imaging in ectopic Cushing syndrome. Current Opinion in Endocrinology, Diabetes & Obesity, 2020, 27(3): 146-154.","DOI":"10.1097\/med.0000000000000541"},{"key":"ref3","doi-asserted-by":"publisher","unstructured":"PAVEL M., \u00d6BERG K., FALCONI M., KRENNING E.P., SUNDIN A., PERREN A., et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 2020, 31(7): 844-860.","DOI":"10.1016\/j.annonc.2020.03.304"},{"key":"ref4","doi-asserted-by":"publisher","unstructured":"HALLET J., LAW C. Role of Primary Tumor Resection for Metastatic Small Bowel Neuroendocrine Tumors. World Journal of Surgery, 2020, 45(1): 213- 218.","DOI":"10.1007\/s00268-020-05727-4"},{"key":"ref5","doi-asserted-by":"publisher","unstructured":"ISIDORI A.M., LENZI A. Ectopic ACTH syndrome. Arq. Bras. Endocrinol. Metab., 2007, 51(8): 1217-1225.","DOI":"10.1590\/s0004-27302007000800007"},{"key":"ref6","doi-asserted-by":"publisher","unstructured":"KATO A., HAYASHI K., NAITOH I., SENO K., OKADA Y., BAN T., et al. Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing\u2019s syndrome. Mol. Clin. Oncol., 2016, 5(1): 113-116.","DOI":"10.3892\/mco.2016.869"},{"key":"ref7","doi-asserted-by":"publisher","unstructured":"SINGER J., WERNER F, KOCH C.A., BARTELS M., AIGNER T., LINCKE T., et al. Ectopic Cushing\u2019s syndrome caused by a well differentiated ACTH- secreting neuroendocrine carcinoma of the ileum. Exp. Clin. Endocrinol. Diabetes, 2010, 118(8): 524-529.","DOI":"10.1055\/s-0029-1243634"},{"key":"ref8","doi-asserted-by":"publisher","unstructured":"DEROOSE C.M., HINDIE E., KEBEBEW E., GOICHOT B., PACAK K., TA\u00cfEB D., et al. Molecular Imaging of Gastroenteropancreatic Neuroendocrine Tumors: Current Status and Future Directions. J. Nucl. Med., 2016, 57(12): 1949-1956.","DOI":"10.2967\/jnumed.116.179234"},{"key":"ref9","doi-asserted-by":"publisher","unstructured":"BIDAKHVIDI N.A., GOFFIN K., DEKERVEL J., BAETE K., NACKAERTS K., CLEMENT P., et al. Peptide Receptor Radionuclide Therapy Targeting the Somatostatin Receptor: Basic Principles, Clinical Applications and Optimization Strategies. 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