Primary Cardiac Lymphoma: Case Series

Abstract

Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is frequently found in immunocompromised patients and is fatal unless timely diagnosed and treated. Untreated, it has a mean survival time of 6 months. The differential diagnosis includes other malignant cardiac neoplasms. Among the imaging modalities, echocardiography is emerging as an indispensable investigation for the early diagnosis and follow-up of these patients, although histopathology gives the final diagnosis. Here, we present three cases of PCL in immunocompetent patients, out of whom two were adults and one was a child. Two patients presented with pericardial tamponade and a third with complete heart block. On echocardiography, lymphoma was located in the right heart chambers in all three cases with the presence of significant pericardial effusion. Histopathological examination showed diffuse large B-cell lymphoma in adults and T-cell lymphoblastic lymphoma in the child. All three patients received chemotherapy. Only two patients survived. The child remained asymptomatic, while the adult male had a relapse after 6 months of completion of the first chemotherapy regimen. The diagnosis of PCL is difficult due to nonspecific clinical manifestations. At present, the main course of treatment is chemotherapy, although surgery may be required in some cases. Among the available imaging modalities, echocardiography is a cost-effective and readily available investigation for the early detection of the tumor.