A cardiac paraganglioma presenting with atypical chest pain

Abstract

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.

Black cardiac paraganglioma in a multiple paraganglioma syndrome

“…The same wise conservative treatment was suggested by Turley et al [8] who partially resected the mass, but was able to ligate the feeding arteries during surgery using colour Doppler on transoesophageal to check the interruption of the blood flow to the tumour. Ali et al [9] preoperatively embolised an intrapericardial pheochromocytoma to facilitate the excision and to limit the catecholamine released during surgical manipulation, but the feeding vessels in this case were not coronary arteries.…”

Right atrial paraganglioma: an unusual primary cardiac tumour

“…Clinical suspicion is raised in cases of resistant hypertension particularly in patients with a family history of phaeochromocytoma. However, more unusual presentations have been reported such as acute myocardial infarction and stroke [3], congestive heart failure [4], hypertensive crisis in labour [5] and upper limb parasthesia [6]. A family history of phaeochromocytoma may indicate MEN2, Von Hippel-Lindau syndrome, neurofibromatosis type 1 and familial phaeochromocytoma-paraganglioma syndrome [7], the latter being the most likely in this case as no features of the other syndromes were present.…”

Surgical treatment of an interatrial septal paraganglioma

“…Because it is difficult to determine whether a paraganglioma is benign or malignant from histopathological examination, rigorous and regular long-term observation is deemed necessary. 2,3,5 Funding Figure 4. Histopathological findings demonstrating the characteristic cell nests (Zellballen pattern).…”