title: Fibrin-associated large B-cell lymphoma: part of the spectrum of cardiac lymphomas.
abstract: Cardiac lymphomas are rare, and the spectrum of pathologic features is not well defined. We encountered an unusual case of cardiac lymphoma residing within a presumed thrombus. To place such cases in context, we reviewed all cardiac lymphomas presenting to a large US cardiovascular medicine referral center during a 30-year period. A total of 14 cardiac lymphomas were identified, and these included 6 primary cardiac lymphomas (PCLs) and 8 lymphomas secondarily involving cardiac structures. Upon review, 3 of the PCLs were confirmed to be diffuse large B-cell lymphoma, not otherwise specified, involving the myocardium. The other 3 cases of PCL lacked myocardial invasion and showed lymphoma cells embedded in fibrin thrombus. Acute inflammation was not evident. These lymphomas presented in immunocompetent male individuals and involved either a prolapsed myxomatous mitral valve, a pseudomyxoma from the left atrium, or a thrombus arising in a synthetic aortic root graft. All 3 consisted of large atypical lymphocytes expressing a nongerminal center B-cell immunophenotype. Two cases were positive for Epstein-Barr virus (latency type III), but none demonstrated human herpes virus-8 latent nuclear antigen. No systemic disease was found at presentation or during follow-up. In our experience, fibrin-associated large B-cell lymphoma arising in the heart represents a substantial proportion of PCL. These lymphomas appear to represent an underrecognized variant of diffuse large B-cell lymphoma with favorable outcome. Further study is needed to understand their natural history.
authors.affiliation: Department of Clinical Pathology, Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
authors.forename: Aaron M
authors.initials: AM
authors.lastname: Gruver
authors.forename: Michael A
authors.initials: MA
authors.lastname: Huba
authors.forename: Ahmet
authors.initials: A
authors.lastname: Dogan
authors.forename: Eric D
authors.initials: ED
authors.lastname: Hsi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003328:Coronary Thrombosis / Q000378:metabolism / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000378:metabolism / Q000473:pathology; D005260:Female; D005337:Fibrin / Q000378:metabolism*; D006338:Heart Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007121:Immunocompetence; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D005337:Fibrin