ECE2014 Poster Presentations Adrenal Medulla (8 abstracts)
A difficult case of ectopic Cushing's syndrome due to ACTH-producing pheochromocytoma presented with normal fractionated urinary catecholamines and metanephrines
Oya Topaloglu 1 , Sefika Burcak Polat 1 , Berna Evranos Ogmen 1 , Husniye Baser 1 , Merve Dirikoc 2 , Mehmet Tokac 3 , Serpil Dizbay Sak 4 , Reyhan Ersoy 1 & Bekir Cakir 1
1Department of Endocrinology and Metabolism, Atatürk Education and Research Hospital, Yildirim Beyazit University, Ankara, Turkey; 2Department of Internal Medicine, Atatürk Education and Research Hospital, Ankara, Turkey; 3Department of General Surgery, Atatürk Education and Research Hospital, Ankara, Turkey; 4Department of Pathology, Faculty of Medicine, Ankara University, Ankara, Turkey.
Introduction: Ectopic ACTH secretion by pheochromocytoma causing Cushing’s syndrome has been reported rarely. Here, we present a patient with ectopic ACTH-dependent Cushing’s syndrome, caused by a pheochromocytoma with normal fractionated 24 h urinary catecholamines and metanephrines.
Case report: A 48-year old female patient admitted to our clinics with the complaints of severe hypertension despite receiving anti hypertensive therapy. Severe headache, malaise, nausea, vomiting, proximal muscle weakness, weight loss and palpitation episodes were the accompanying symptoms. In the physical examination, she was anxious and depressive. Her pulse was 140/min, blood pressure was 200/100 mmHg. She didn’t have any typical sign of Cushing syndrome except proximal myopathy. Labaratory tests were significant for severe hypokalemia (2.5 mmol/l), hypoalbuminemia (2.6 g/dl). Her serum cortisol concentration was 63.4 μg/dl and it wasn’t supressed after 1 mg and 2 days 2 mg dexamethasone suppression tests (DST). 24 h urinary free cortisol was extremely high (18980 μg/day) whereas urinary fractionated catecholamines and metanephrines were normal. Her serum ACTH was measured with IRMA and was found as 289.3 pg/ml. Our initial endocrinologic diagnosis was ACTH- dependent ectopic cushing syndrome since there wasn’t any supression with HDDST, treatment resistant hypokalemia, negative pitiutary imaging and dramatic and rapidly progressing clinics. A computed tomography scan revealed a 43×37 mm tumoral mass in the left adrenal gland and PET-CT showed a 5 cm sized lesion in the left adrenal gland with high SUVmax (18.7) and hyperplasia in the other adrenal. She was underwent right adrenalectomy with Addison and pheochromocytoma protocol. After operation pathological examination was compatible with pheochromocytoma, and immunostained with ACTH extensively.
Conclusion: Ectopic ACTH-secreting pheochromocytoma is a diagnostic challenge for the clinicians. Serum or urinary metanephrines and catecholamines are very sensitive and specific tests for the diagnosis of pheochromocytoma but negative results may occur and confusion in the diagnosis.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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