Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle via a glucocorticoid-driven positive-feedback mechanism

Endocrine Journal
Minako InoueTakanari Kitazono

Abstract

In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hypercatecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH. Moreover, his ACTH/cortisol ratio and catecholamine levels were extremely high, suggesting catecholamine-dominant ACTH-secreting pheochromocytoma. Computed tomography revealed a large right adrenal tumor. 18F-FDG positron emission tomography showed uptake in the area of the adrenal tumor, while 123I-metaiodobenzylguanidine scintigraphy showed no accumulation. His plasma ACTH level paradoxically became elevated after a dexamethasone suppression test. After metyrapone administration, not only serum cortisol but also plasma ACTH levels were exponentially decreased almost in parallel, suggesting a glucocorticoid-driven positive-feedback regulation in this rapidly exacerbate...Continue Reading

References

Jul 23, 1977·British Medical Journal·W J JeffcoateG M Besser
Aug 23, 1979·The New England Journal of Medicine·R F SparkL Landsberg
Mar 1, 1992·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·R Chan, M F Michelis
Oct 1, 1987·The American Journal of the Medical Sciences·H SakuraiT Fu
Jul 18, 1968·The New England Journal of Medicine·T S HarrisonR S Wojtalik
Nov 1, 1984·Acta Pathologica Japonica·K KakudoM Fukuchi
Sep 1, 1982·Annals of Surgery·C J DaviesR B Welbourn
Apr 1, 1984·Journal of Endocrinological Investigation·J O SchroederR Volpé
Dec 1, 1994·Journal of Endocrinological Investigation·M TerzoloA Angeli
Aug 1, 1993·Clinical Endocrinology·A White, A J Clark
Oct 1, 1996·European Journal of Endocrinology·K C LohA H Shlossberg
Jun 18, 1998·Internal Medicine·M SatoH Kitamura
May 18, 1999·Cardiovascular Research·M E Ullian
Dec 22, 1999·European Journal of Endocrinology·W K KhalilL Gaspar
Aug 3, 2002·European Journal of Endocrinology·P Sytze van DamWouter W de Herder
Jun 6, 2003·The Journal of Clinical Endocrinology and Metabolism·Marcus Quinkler, Paul M Stewart
Jan 8, 2004·Endocrine Journal·Hyoung Chul OhWon Bae Kim
May 26, 2005·The Journal of Clinical Endocrinology and Metabolism·Ioannis IliasLynnette K Nieman
Aug 16, 2005·The Medical Journal of Australia·Yaser AlderaziStan B Sidhu
Aug 23, 2005·Lancet·Jacques W M LendersKarel Pacak
Aug 17, 2006·The Journal of Clinical Endocrinology and Metabolism·Frederieke M BrouwersKarel Pacak
Oct 26, 2006·European Journal of Endocrinology·Luiz Roberto SalgadoBerenice Bilharinho de Mendonça
Jul 27, 2007·The Journal of Clinical Endocrinology and Metabolism·Laurence AmarPierre-François Plouin
Feb 27, 2008·European Journal of Endocrinology·Alejandro L RosasGraeme Eisenhofer
Apr 17, 2008·The Journal of Clinical Endocrinology and Metabolism·B M K BillerM Boscaro
Jul 11, 2009·Gynecological Endocrinology : the Official Journal of the International Society of Gynecological Endocrinology·Clémentine CohadePhilippe Caron
Oct 13, 2009·European Journal of Internal Medicine·M F NijhoffA M Pereira
Dec 5, 2009·Journal of the American College of Surgeons·Marlon A GuerreroQuan-Yang Duh
Apr 16, 2010·The Journal of Clinical Endocrinology and Metabolism·Arnold F JacobsonRichard R Black
Jun 11, 2010·Indian Journal of Urology : IJU : Journal of the Urological Society of India·Meyyappan M RamasamyPravin S Dass
Mar 1, 2012·World Journal of Surgery·James Kirkby-BottBruno Carnaille
Mar 8, 2012·Endocrine·Chitrabhanu BallavAshley B Grossman
May 31, 2012·Endocrine Pathology·Maria Francesca CassarinoFrancesca Pecori Giraldi
Jan 4, 2013·The Journal of Clinical Endocrinology and Metabolism·Anouk ScholtenQuan-Yang Duh
Jan 9, 2014·Gynecological Endocrinology : the Official Journal of the International Society of Gynecological Endocrinology·Baoyou HuangFeiyun Zheng
Jun 4, 2014·The Journal of Clinical Endocrinology and Metabolism·Jacques W M LendersWilliam F Young

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Citations

Mar 2, 2019·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Caitlin LynchMenghang Xia
Jul 28, 2020·The Journal of Clinical Endocrinology and Metabolism·Patrick F ElliottHenrik Falhammar
Jun 2, 2021·Journal of the Endocrine Society·Yasutaka TsujimotoKazuo Chihara

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