Sunday, October 11, 2009

ACTH-producing pheochromocytoma: Clinical considerations and concise review of the literature

M.F. Nijhoffab, O.M. Dekkersac, L.J. Vlemingb, J.W.A. Smita, J.A. Romijna, A.M. PereiraaCorresponding Author Informationemail address

Received 5 May 2009; received in revised form 9 July 2009; accepted 10 August 2009. published online 01 September 2009.


We present a patient with a rare cause of ectopic ACTH-dependent Cushing's syndrome, caused by a pheochromocytoma. The case provides clues for a detailed discussion on the pitfalls and diagnostic difficulties in establishing the correct underlying cause of ACTH-dependent Cushing's syndrome. It clearly demonstrates that the relative contributions of clinical, biochemical, and radiological clues in establishing the correct underlying cause of Cushing's syndrome may differ considerably between Cushing's disease and Cushing's syndrome due to ectopic overproduction of ACTH. In addition, the literature concerning ACTH-producing pheochromocytomas is reviewed.


Keywords: Pheochromocytoma, Adrenocorticotropic hormone, Cushing's syndrome, ACTH syndrome, Ectopic

a Department of Endocrinology & Metabolism, C4-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands

b Department of Internal Medicine, HAGA, location Leyenburg, Leyweg 2752545 CH the Hague, The Netherlands

c Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands

Corresponding Author InformationCorresponding author. Tel.: +31 71 6263082; fax: +31 71 524 8146.

PII: S0953-6205(09)00159-9


© 2009 European Federation of Internal Medicine. Published by Elsevier Inc All rights reserved.



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