Cardiac paraganglioma: Clinical presentation, diagnostic approach and factors affecting short and long-term outcomes

Background: We describe our experience of the case of a young female who presented with chest pain and was found to have an intra-pericardial mass that was later confirmed to be a paraganglioma. Although preoperative magnetic resonance imaging (MRI) did not show any left atrial invasion, the patient died in the peri-operative period due to irreparable damage of the cardiac structures from extensive neoplastic invasion. We then performed a retrospective analysis of the available literature to find the factors associated with adverse surgical and long-term outcomes in patients with cardiac paraganglioma. Methods: We found 93 case reports of cardiac and/or pericardial paragangliomas in the literature. After exclusions, 82 cases were included in the final analysis. The patients were divided into two groups based on the outcome of surgical management. Univariate analysis was performed using SPSS software (Chicago, IL version 18), and the statistical significance was defined as a p-value < 0.05. Results and conclusions: The comparison of available demographic, clinical, pathological and laboratory parameters between the deceased and the surviving patients revealed that only the intra-cardiac location (p-value = 0.021) and the development of metastases (p-value < 0.001) were independently associated with increased surgical and long-term mortality, respectively. The size of a paraganglioma, its functional status or invasion into the surrounding structures does not appear to affect short-or long-term survival in these patients. The Kaplan-Meier survival curve showed excellent long-term prognosis for patients with a complete surgical removal of the neoplasm. Based on our experience, we also suggest preoperative imaging with a three dimensional cardiac CT and evaluation for cardiac transplantation before embarking on the surgical resection of these tumors.