Primary lymphoma of the heart: Case report and literature review

Abstract

Primary cardiac lymphoma (PCL) is a rare and usually fatal neoplasma. A case of PCL in a 78-year-old man who complained of exertional dyspnea and peripheral edema is presented. Echocardiography revealed a mass in the right atrium and a diagnosis of low-grade B-cell lymphoma was obtained with the surgically resected tumor. The lesion appeared to have originated in the right atrium and involved the right ventricle. The patient died of bronchopneumonia 8 months after the initial consultation. The present case and 39 patients with PCL reported between 1995 and 2002 were reviewed. Forty patients showed various and non-specific symptoms such as dyspnea, edema, arrhythmia and pericardial effusion. Primary cardiac lymphoma occurred slightly more often in male patients (M : F = 23:17) and in the elderly in general (mean age, 67 years), with lesions found in the following locations, listed in order of frequency: right atrium, pericardium, right ventricle, left atrium, left ventricle, and other sites. Antemortem diagnosis was obtained in 37 of the 40 patients. Thirty-seven cases were of B-cell lineage and two cases were of T-cell lineage. Complete remission was obtained in only 15 of the 40 patients. Although PCL antemortem diagnoses have been made in the majority of recent cases, the prognosis still remains poor.

Characteristics and Survival of Malignant Cardiac Tumors

“…25 Previous reports show that diffuse large B-cell lymphomas have a predilection for the right side of the heart (92% had involvement of right atrium or right ventricle) and usually present with dyspnea, constitutional symptoms, pain and arrhythmias. 25,[27][28][29] About 90% of patients with diffuse large B-cell lymphomas receive a anthracycline-based regimen, with high treatment-related mortality. 25 Historically, ≈28% of patients are treated with surgery and 20% with radiation, slightly higher than what we found at 16.5% and 15.1%, respectively.…”

Primary Cardiac B-cell Lymphoma in Both Atria

“…In the present case, cardiac B-cell lymphoma appeared as diffusely thickened atria, which was found by routine echocardiographic examination. Primary cardiac lymphoma commonly appears as mass formation and rarely appears as a hypertrophic ventricular wall [1]. There has been only one case report of primary cardiac lymphoma with diffusely thickened atria [2], and to our knowledge, this is the second case of cardiac lymphoma with a hypertrophic atrial wall.…”

High-dose chemotherapy with autologous stem cell transplantation following systemic chemotherapy, prophylactic intrathecal methotrexate, and radiotherapy prevents relapse and improves the outcome of advanced stage primary testicular lymphoma even with cardiac involvement

“…6,7 As the rate of complete remission (CR) for primary cardiac lymphoma is only 37.5%, it has a poor prognosis. 8 Similarly, secondary cardiac lymphoma patients also have a very poor outcome due to the high risk of sudden death. Most patients die before the start of therapy.…”