Primary cardiac lymphoma with isolated parenchymal central nervous system relapse: report of two cases and review of the literature

Abstract

Primary cardiac lymphoma (PCL) is a rare subset of non-Hodgkin’s lymphoma involving the heart and/or pericardium with no or minimal evidence of extracardiac involvement at presentation. Distant relapses have infrequently been observed.We report two cases of this disorder that showed isolated central nervous system (CNS) relapse. Diagnosis by endomyocardial biopsy was consistent with diffuse large B-cell lymphoma. After immunochemotherapy they achieved complete remission (CR). Eight and five weeks after, isolated CNS relapses were observed respectively. The first patient was treated with high-dose methotrexate (HD-MTX) and high-dose cytarabine, resulting in a second CR. She then went onto receive autologous stem-cell transplantation but unfortunately died shortly after because of infection. The second patient received systemic CNS prophylaxis with HD-MTX, and later she was treated with an induction chemotherapy strategy with evidencing of progressive disease after two courses of treatment. She was subsequently initiated on a salvage therapy with cytarabine, followed by whole-brain radiotherapy, and autologous stem-cell transplant (ASCT), finally achieving a complete remission.Isolated CNS relapse is a very uncommon pattern in PCL and a standard approach to treatment is not yet well established. Nevertheless, the importance of CNS evaluation, using magnetic resonance imaging (MRI) and lumbar puncture, in patients with PCL should be considered, and further studies are recommended to determine the appropriate management of this complication.

Primary Cardiac Lymphoma: Lessons Learned from a Long Survivor

“…Rarely, isolated extranodal and extracardiac relapse, for example, in the central nervous system, have been reported [21, 22]. Our patient relapsed after 7 years with an isolated breast lesion.…”

“…A few cases of early relapse have been described in the literature [5, 21]. Rarely, isolated extranodal and extracardiac relapse, for example, in the central nervous system, have been reported [21, 22].…”

Case Report: Cardiac Involvement by Lymphoma: Rare but Heterogeneous Condition With Challenging Behaviors

“…A few case reports (17-21) described isolated parenchymal CNS relapse in patients with PCL: in most cases, similarly to what happened in our patient, relapsed occurred shortly after the completion of chemotherapy (within two months) in responders. Only one patient had received CNS prophylaxis with intermediate-dose (1.5 g/m 2 ) methotrexate, administered every two weeks for four doses (20). A proportion of these patients responded to second-line therapy, but long-term followup is missing.…”

Primary cardiac lymphoma with central nervous system relapse

“…Our two patients developed a CNS relapse at 6 months and 2 years from initial diagnosis. We identified six additional PCL cases in the literature, who also developed isolated CNS recurrence of the lymphoma . CNS disease typically developed within a few months after initial presentation, raising the question whether occult CNS localization was already present at the time of diagnosis.…”