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Exp Clin Endocrinol Diabetes 2006; 114(8): 444-447
DOI: 10.1055/s-2006-924154
Case Report

© J. A. Barth Verlag in Georg Thieme Verlag KG · Stuttgart · New York

Cushing's Syndrome Due to Ectopic CRH Secretion by Adrenal Pheochromocytoma Accompanied by Renal Infarction

F. Bayraktar 1 , L. Kebapcilar 1 , M. A. Kocdor 2 , S. L. Asa 3 , S. Yesil 1 , S. Canda 4 , T. Demir 1 , A. Saklamaz 1 , M. Seçil 5 , B. Akinci 1 , S. Yener 1 , A. Comlekci 1
  • 1DEU (Dokuz Eylul University), Department of Internal Medicine, Division of Endocrinology and Metabolism, Izmir, Turkey
  • 2DEU, Department of Surgery, Izmir, Turkey
  • 3University of Toronto, Department of Laboratory Medicine and Pathobiology, Toronto, Ontario, Canada
  • 4DEU, Department of Pathology, Izmir, Turkey
  • 5DEUTF, Department of Radiology
Further Information

Publication History

Received: September 23, 2005

Accepted: April 3, 2006

Publication Date:
13 October 2006 (online)

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Abstract

Ectopic production of corticotropin-releasing hormone (CRH) by a pheochromocytoma is an infrequent cause of Cushing's syndrome. We report the case of a 43-year-old man with Cushing's syndrome due to a CRH-producing adrenal pheochromocytoma. The patient had clinical and biochemical evidence of hypercortisolism in conjunction with high ACTH levels and non-suppressible serum cortisol levels on low-dose and high-dose dexamethasone suppression testing. In addition to these clinical features of one month's duration, the patient developed symptoms of pheochromocytoma including headache, hypertension that was resistant to conventional therapy and excessive sweating. Biochemical testing confirmed elevated 24-hour urinary catecholamines and metabolites. Abdominal CT revealed a 4.5 × 4 × 3.5 cm mass in the left adrenal gland. He underwent elective left adrenalectomy. Light microscopic and immunochemical studies revealed a pheochromocytoma that contained immunoreactive CRH and was negative for ACTH. Plasma ACTH and dexamethasone supression tests normalized after surgery. This is an unusual case of a CRH-secreting pheochromocytoma. This was complicated by renal infarction, illustrating further the complexity of Cushing's syndrome in a patient with pheochromocytoma caused by CRH hypersecretion.

Key words

Pheochromocytoma - ectopic CRH secretion - Cushing's syndrome - hypertension

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Correspondence

Levent KebapcilarMD 

Dokuz Eylul University·Department of Internal Medicine· Division of Endocrinology and Metabolism

123/4 Street No: 17/8 Hatay/IZMIR

Izmir

Turkey

Phone: +90/23/22 85 63 71

Fax: +90/23/22 59 11 38

Email: leventkebapcilar@yahoo.com

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