Primary cardiac lymphoma

Primary cardiac lymphoma (PCL) is a very rare disorder. Histologically, the majority of cases of PCL are diffuse B-cell lymphoma. PCL occurs more frequently in immunocompromised patients. Symptoms may vary according to the heart site involved. The most frequent cardiac clinical manifestations associated with PCL are pericardial effusion, heart failure, and atrioventricular block (AV-block). Diagnosis of PCL can be suggested by transesophageal echocardiography, computed tomography, and magnetic resonance imaging. However, cytologic examination of cardiac tumor or pericardial effusion is paramount for a definite diagnosis of this condition. Prognosis of PCL is poor with a median survival of 7 months after initial diagnosis. Newer modalities including immunotherapy with rituximab or auto stem cell transplantation are promising in the treatment of this lethal condition.