P139 CARDIAC PARAGANGLIOMA: THE IMPORTANCE OF MULTIMODALITY IMAGING

Abstract

Abstract Cardiac paraganglioma is a rare neuroendocrine tumour secreting norepinephrine and causing severe hypertension, palpitations, tachyarrhythmias; its precise location is very important because the compression of cardiovascular structures may result in ischaemia, arrhythmia and cardiac failure. Surgical resection represents the primary treatment modality. A 40–year–old young woman with history of hypertension, nocturnal tachycardia and sweats with elevated levels of urinary metanephrine underwent abdominal MRI angiography showing normal kidneys and adrenal glands while in the cardiac–mediastinal site a solid neoformation with contrast impregnation confirmed at subsequent chest CT scan with contrast medium and it appeared inseparable from superior vena cava and occupying the atrial chambers. Patient started therapy with an α–adrenoceptor blocker and underwent cardiac MRI documenting the intrapericardial site of the lesion infiltrating atrial septum and PET/CT scan revealing a large area of uptake of the analogue of somatostatin in the mediastinum typical for atrial paraganglioma. Subsequent angiography CT scan of the thoracic aorta and coronary CT scan showed no signs of infiltration on the great vessels and heart chambers but there was not a clear adipose cleavage plane and the mass appeared intensely vascularized by two large vessels arising from the circumflex artery and the isthmic aorta. After specialistic surgical evaluation the patient was treated with ligation of the afferent peduncle to the neoformation and biopsy of the tissue sample was performed. Post–procedure angiography CT scan showed the neoformation unchanged in size with the two unchanged afferent branches. Histological examination of the tissue sample concluded with arteriovenous hemangioma. One month after surgery elevated urinary normetanephrine levels persisted and the mass on echocardiogram was unchanged. Cardiac MRI after three months was unchanged and specialistic surgical evaluation did not suggest reoperation. Six months after surgery cardiac MRI showed mild increase in volume of the intrapericardial lesion and unchanged atrial septum infiltration. The multimodality imaging plays an important role in the diagnosis of cardiac paraganglioma. The arterial ligation, without the completely removal of the lesion, was not conclusive, the mass increased in size and the patient remained symptomatic. Surgical resection is necessary for the complete cure of the syndromes.