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

Abstract

Cardiac lymphomas are rare extranodal lymphomas involving primarily and secondarily the heart and/or pericardium. Here we describe three cases of cardiac involvement from lymphoma with specific peculiarities: two primary cardiac Diffuse Large B-cell Lymphomas and one secondary involvement from Marginal Zone Lymphoma (MZL). The first case highlights the issue of early CNS relapse and the possible role for CNS prophylaxis; the second case demonstrates the difficulties of interpretation and possible mistakes of different radiologic techniques adopted to evaluate cardiac involvement by lymphoma during follow-up; the third is a unique case of MZL with cardiac involvement. Our aim is to share the findings observed in these cases putting them in relation with data from the literature.

Keywords: MRI; PET/CT (18)F-FDG; central nervous system relapse/progression; diffuse large B cell lymphoma; heart involvement; lymphoma; marginal zone lymphoma; primary cardiac lymphoma.

Figure 1

CT-PET abnormal 18-FDG uptake on right ventricle thickening 3 months (SUV 4.9, DS 4) (A) and 18 months (SUV 5 – DS 4) (B, C) after ASCT. Cardiac MRI images of June 2019 (from D to F) show the persistence of the right ventricle anterior wall thickening (14x56 mm). (D) Short tau inversion recovery (STIR) T2 black blood image in short axis on the cardiac base. (E) Steady-state free precession (SSFP) balance image with T2/T1 weighting in short axis on the cardiac base. (F) IR TSET1 image for evaluation of myocardial late enhancement in short axis on the cardiac base.

Figure 2

Decrease in cardiac wall thickness after chemotherapy at echocardiographic evaluation (from A to F). (A, C) interventricular septum (IVS) and posterior wall (PW) thickness, 18 mm and 15 mm respectively, from parasternal long-axis and short-axis view, before chemotherapy. (B, D) IVS and PW thickness, 12 mm and 10 mm, respectively, from parasternal long-axis and short-axis view, after chemotherapy. (E, F) right ventricular lateral wall (RV-LW) thickness, 9 mm and 6 mm, respectively, from 4 chamber view; of note, pericardial effusion decreases from 18 mm to 6 mm after chemotherapy. (G) monoclonal IgM/K before starting treatment. (H) no more detectable monoclonal paraprotein after treatment. (I) histologic sample of endomyocardial biopsy, hematoxylin-eosin staining.