Endocrine Abstracts (2006) 11 P92

Purely adrenaline-secreting phaeochromocytoma: a classical presentation of a rare entity

A Merghani1, KH Darzy1, D Morris1, R Carpenter2, A Deaner3 & JP Monson1

1Department of Endocrinology, St. Bartholomew’s Hospital, London, United Kingdom; 2Department of Surgery, St. Bartholomew’s Hospital, London, United Kingdom; 3Department of Cardiology, King George Hospital, London, United Kingdom.

A provisional diagnosis of paroxysmal atrial fibrillation with normal blood pressure and echocardiogram was made in a 58 years old man. He described ‘funny turns’ on standing during which he would become sweaty, tremulous with palpitations and headache and facial flushing preceded by pallor. The patient was otherwise fit and healthy with a BMI of 27.3 kg/m2; he used to drink about 15 pints of beer per week and smoke 11 cigarettes daily for the last 40 years, both stopped a year after initial diagnosis. General and systematic examination was normal and he had no significant past medical history or relevant family history. Initial treatment with beta-blockers failed with increasing frequency of these episodes (1–2 per month). Flecainide was then introduced, which provided partial symptomatic improvement in terms of severity of the attacks although the frequency increased. In view of this, urinary catecholamines were requested and demonstrated increased adrenaline of 252 and 196 nmol/24 h (normal range, 0–144 nmol/24 h) but persistently normal noradrenaline and dopamine levels. Abdominal CT scanning revealed a 3.4 cm enhancing heterogeneous mass in the left adrenal gland, the radiological characteristics of which were in keeping with phaeochromocytoma. An incidental benign looking renal cyst was found but the patient had normal brain MRI and no retinal angioms, thus excluding VHL syndrome. MIBG scanning showed intense uptake in the left adrenal. Treatment with alpha blockade (phenoxybenzamine 10 mg qds) and beta blockade (propranalol 40 mg tds) produced complete symptomatic improvement with no objective fall in BP. The patient underwent uncomplicated left adrenalectomy following standard preparation. Histology showed a well encapsulated and completely excised phaeochromocytoma. The treatment was stopped after surgery and the patient remained very well and symptom-free with normal urinary catecholamines. Pure adrenaline secreting phaeochromcytoma is rare. This case highlights the importance of considering this rare entity in patients with paroxysmal symptoms/cardiac dysrhythmias in the absence of hypertension or cardiac pathology.

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