Primary Cardiac Lymphoma: Report of Two Cases Occurring in Immunocompetent Subjects

Abstract

Primary cardiac lymphomas (PCLs), involving solely heart and/or pericardium at presentation, are rare events. They are frequently recognized at autopsy and generally carry a poor prognosis due either to a delay in the diagnosis or to infiltration of heart structures. We report here on two patients with large B-cell PCL. One is a 52-year-old man who presented with multiple cardiac tumors infiltrating mainly the right atrium and the inter-atrial septum. Diagnosis was established by ultrasound-assisted transesophageal biopsy of the intra-atrial multilobated tumor mass. He was treated with Rituximab-implemented high-dose sequential (R-HDS) chemotherapy followed by autologous peripheral blood stem cell transplantation, attaining complete response. He had no evidence of disease 24 months from onset. The second patient was a 70-year-old woman who presented with pericardial tamponade and low-output cardiac failure. Despite prompt pericadiocentesis and chemotherapy with cyclophosphamide and vincristine, she died 2 weeks later. Postmortem examination revealed large B-cell lymphoma proliferation confined to the heart. Whether primitive heart localizations represent an independent prognostic factor, and what specific measures should be adopted in patients with this rare presentation is the subject of the present report and review of the literature.

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 lymphoma: Utility of multimodality imaging in diagnosis and management

“…Once the diagnosis of PCL has been established, the literature is quite limited in terms of recommendations for monitoring treatment response in PCL. 7,8,11 The paucity of monitoring recommendations may be due in part to the radiographic heterogeneity seen between different PCL cases when comparing the same imaging technique and between imaging techniques used to characterize a single PCL case. Each modality has limitations that are important to note.…”

“…Chemotherapy, immuno-chemotherapy, stem cell transplant, radiation therapy and combined modality approaches have been used to treat PCL. 7,8,[11][12][13][14] Recent reviews have attempted to assess CR rates using these various approaches in PCL. 7,8 Traditionally, PCL is thought to have an extremely poor prognosis due to life-threatening complications, such as cardiac rupture, which can occur at diagnosis or during treatment due to the anatomical location of the tumor.…”

Primary Cardiac Lymphoma in an Immunocompetent 71-Year-Old Man

“…To our knowledge, only 23 cases of PCL in immunocompetent patients have been reported to date (Table I). [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] According to these reports, no patient presented with noncardiac lymphomatous localization, but, as in our patient, it was impossible to absolutely exclude noncardiac involvement in the absence of autopsic investiga- Nine of these patients were treated with surgical excision followed by chemotherapy, and 5 of them survived at least 6 months after treatment. No evidence exists concerning the necessity of surgery as an adjunct to chemotherapy in PCL; moreover, in those 9 cases surgery was the final diagnostic step, along with excision as treatment.…”