A 62-year old man was referred for severe but asymptomatic hypertension. His background was significant for extensive peripheral vascular disease, heavy smoking and hypercholesterolemia. His blood pressure was 218/100 mmHg despite verapamil SR and hydralazine. There were bruits over the abdominal aorta, carotid and renal arteries. He had mildy impaired renal function (serum creatinine 102 μmol/L) and normal serum electrolyte levels. His renin and aldosterone were 232 mU/L (normal 3-40) and 2270 pmol/L (normal 100-950 ), respectively while temporarily on spironolactone. A renal artery duplex ultrasound (RADU) showed an increased peak systolic velocity in the left renal artery of 640 cm/sec (normal <180 cm/sec) with a renal/aortic velocity ratio of >10 (normal <3.5).
Unexpectedly, his noradrenaline and normetadrenaline levels came back significantly elevated (20 nmol/L (normal <3.5) and 13000 pmol/L (normal <900), respectively). Subsequent 123I-metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) scan revealed a large avid lesion in the posterolateral bladder wall and a small pelvic wall lymph node. A CT-angiogram demonstrated a high-grade stenosis of the left renal artery. A Technetium-99m-mercaptoacetyltriglycine (99Te-MAG3) renal scan showed an atrophic left kidney only contributing 13% to overall function.
Phenoxybenzamine and irbesartan were added to his treatment. He has been proposed for a cystoprostatectomy, pelvic lymph node clearance and ileal conduit formation. A simultaneous left nephrectomy is under consideration.
This is a case of severe hypertension explained by a rare combination of a bladder paraganglioma and possibly also renal artery stenosis (RAS). Physicians will frequently be asked to assess hypertensive patients for secondary causes. Well-known causes include primary aldosteronism, RAS, obstructive sleep apnoea and phaeochromocytoma/paraganglioma. The choice of diagnostic tests is often guided by the clinical presentation. Our patient, however, illustrates the importance of a systematic and comprehensive approach as characteristic clinical manifestations may be absent and occasionally more than one cause present.