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An intrapericardial pheochromocytoma presented with paroxysmal attacks of hypertension and palpitation: typical presentation with unusual location of the tumour
  1. Y-G Ko,
  2. J-W Ha,
  3. B-C Chang

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A 60 year old man came to the emergency room because of severe chest pain, headache, and palpitations accompanied by facial flushing and sweating. On admission his blood pressure was 200/100 mm Hg, and pulse rate 110 beats/min. ECG showed no specific findings for myocardial ischaemia. A computed tomography scan to rule out aortic dissection showed, however, a 8 × 5 cm sized dumbbell shaped mass within the pericardial space between the aorta and the main pulmonary artery. The mass abuts the ascending aorta, main pulmonary artery, and left main and proximal left anterior descending artery; however, invasion or adhesion was excluded by demonstrating sliding motion between the mass and the adjacent structures with cine magnetic resonance imaging (MRI) (panels A, B). A 24 hour urine study revealed raised concentrations of noradrenaline (norepinephrine) (233.7 μg/day, normal range 15–80 μg/day) and vanillynmandelic acid (23.29 mg/day, normal range < 8 mg/day). Abdominal MRI showed, however, no adrenal mass. A [131I]metaiodobenzyl-guanidine scintiscan revealed a hot uptake spot anatomically corresponding to the intrapericardial mass (panel C). The dumbbell shaped mass between the aorta and the main pulmonary artery was successfully excised (panel D) under cardiopulmonary bypass without damaging adjacent vessels after treatment with phenoxybenzamine and propranolol for four weeks.

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Intrapericardial pheochromocytoma. Panels A, B: cardiac magnetic resonance image of dumbbell shaped mass (asterisk) between the aorta and the main pulmonary artery. Panel C: hot uptake spot (white arrow) in [131I]metaiodobenzyl-guanidine scintiscan. Panel D: gross appearance. Panel E: histologic appearance of the tumour strongly immunoreactive for synaptophysin and chromogranin. Ao, aorta; MPA, main pulmonary artery.