A cardiac paraganglioma presenting with atypical chest pain

Primary cardiac tumours are rare. The majority are benign and 75% are atrial myxomas. One of the more unusual benign tumours affecting the heart is a cardiac paraganglioma. A 56-year male was presented with a 6-month history of vague, left-sided chest pain, intermittent parathesia of the left arm and dyspnoea on bending. Echocardiography documented a large, highly vascular mass, attached to the intra-atrial septum. All investigations, haematological, biochemical, neuroendocrine tumour markers and urinary cathecholamine levels, were within normal limits. Macroscopically the tumour involved the whole of the atrial septum, roof of the left atrium and extended to surround the superior vena cava, excluding total resection. The feeding vessels were ligated in the hope of infarcting the remainder of the tumour. In over 2-years of follow-up, the patient remains clinically stable. To our knowledge this is the first reported case where vascular ligation has been used to control a cardiac paraganglioma.