An unusual case of primary cardiac prosthetic valve-Associated lymphoma

Abstract

Primary cardiac tumors are extremely rare neoplasms with an incidence of less than 0.4%.1-3 Primary cardiac lymphoma (PCL), the majority of which is non-Hodgkin lymphoma, accounts for around 2% of cardiac tumors and less than 0.5% of extranodal lymphomas.1,4-6 Primary lymphoma involving cardiac valves has been described in few case reports and small case series owing to its rarity.7-10 Most cases of PCL present with manifestations of congestive heart failure or cardiac arrhythmias,11 whereas primary valve-associated lymphoma (PV-AL) is usually diagnosed incidentally during valve repair or replacement. The pathophysiology remains unclear, but a few cases have been associated with Epstein Barr virus (EBV).7 Cases previously described in the literature carried an overall poor prognosis and to date there is no standardized treatment approach. We provide here an unusual case of primary prosthetic valve-associated cardiac large B-cell lymphoma, which was successfully treated with adjuvant chemotherapy after valve repair and which resulted in an excellent long-term outcome.