A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome
Keywords:ACTH secreting Tumors, Duodenal carcinoid, Ectopic Cushing’s syndrome
ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years. Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.
Lacroix A, Feelders RA, Stratakis CA, Nieman LK. Cushing’s syndrome. Lancet Lond Engl. 2015 Aug 29;386(9996):913-27.
Ferraù F, Korbonits M. Metabolic comorbidities in Cushing’s syndrome. Eur J Endocrinol. 2015 Oct;173(4):M133-157.
Nieman LK, Biller BMK, Findling JW, Newell-Price J, Savage MO, Stewart PM, et al. The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40.
Arnaldi G, Angeli A, Atkinson AB, Bertagna X, Cavagnini F, Chrousos GP, et al. Diagnosis and complications of Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab. 2003 Dec;88(12):5593-602.
Biller BMK, Grossman AB, Stewart PM, Melmed S, Bertagna X, Bertherat J, et al. Treatment of adrenocorticotropin-dependent Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab. 2008 Jul;93(7):2454-62.
Gardner DG, Greenspan FS, editors. Greenspan’s basic & clinical endocrinology. 9. ed. New York: McGraw Hill Medical; 2011. 880 p.
Williams R. Williams textbook of endocrinology. 13th edition. Melmed S, Polonsky KS, Larsen PR, Kronenberg H, editors. Philadelphia, PA: Elsevier; 2016. 1916 p.
Tani Y, Sugiyama T, Hirooka S, Izumiyama H, Hirata Y. Ectopic ACTH syndrome caused by bronchial carcinoid tumor indistinguishable from Cushing’s disease. Endocr J. 2010;57(8):679-86.
Zemskova MS, Gundabolu B, Sinaii N, Chen CC, Carrasquillo JA, Whatley M, et al. Utility of various functional and anatomic imaging modalities for detection of ectopic adrenocorticotropin-secreting tumors. J Clin Endocrinol Metab. 2010 Mar;95(3):1207-19.
Aniszewski JP, Young WF, Thompson GB, Grant CS, van Heerden JA. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion. World J Surg. 2001 Jul;25(7):934-40.
Uecker JM, Janzow MT. A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum. Am Surg. 2005 May;71(5):445-6.
Grossrubatscher E, Vignati F, Dalino P, Possa M, Belloni PA, Vanzulli A, et al. Use of radioguided surgery with [111In]-pentetreotide in the management of an ACTH-secreting bronchial carcinoid causing ectopic Cushing’s syndrome. J Endocrinol Invest. 2005 Jan;28(1):72-8.
Beuschlein F, Hammer GD. Ectopic pro-opiomelanocortin syndrome. Endocrinol Metab Clin North Am. 2002 Mar;31(1):191-234.
Waisberg J, Joppert-Netto G, Vasconcellos C, Sartini GH, Miranda LSV de, Franco MIF. Carcinoid tumor of the duodenum: a rare tumor at an unusual site. Case series from a single institution. Arq Gastroenterol. 2013 Mar;50(1):3-9.
Nakao Y, Tsuruzawa M, Kanaoka H, Tanioka K, Sudo M. [Cushing’s syndrome caused by ACTH-producing carcinoid tumor originating in the duodenum]. Horumon To Rinsho. 1975 Jun;23(6):603-7.
Khare J, Daga S, Nalla S, Deb P. Ectopic adrenocorticotropic hormone syndrome in a case of duodenal neuroendocrine tumor presenting with liver metastasis. J Postgrad Med. 2018;64(1):47-9.