Cushing's syndrome due to ectopic CRH secretion by adrenal pheochromocytoma accompanied by renal infarction

• Published in: Experimental and clinical endocrinology & diabetes Vol. 114; no. 8; p. 444
• Main Authors: Bayraktar, F, Kebapcilar, L, Kocdor, M A, Asa, S L, Yesil, S, Canda, S, Demir, T, Saklamaz, A, Seçil, M, Akinci, B, Yener, S, Comlekci, A
• Format: Journal Article
• Language: English
• Published: Germany 01.09.2006
• Subjects: Adrenal Gland Neoplasms - diagnosis; Adrenal Gland Neoplasms - diagnostic imaging; Adrenal Gland Neoplasms - pathology; Adrenal Gland Neoplasms - surgery; Adrenocorticotropic Hormone - blood; Adrenocorticotropic Hormone - secretion; Corticotropin-Releasing Hormone - secretion; Cushing Syndrome - etiology; Humans; Hypertension - etiology; Infarction - diagnosis; Male; Middle Aged; Pheochromocytoma - diagnosis; Pheochromocytoma - diagnostic imaging; Pheochromocytoma - pathology; Pheochromocytoma - surgery; Renal Circulation; Tomography, X-Ray Computed; Treatment Outcome
• ISSN: 0947-7349
• DOI: 10.1055/s-2006-924154

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 x 4 x 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.