Fibrin-associated Large B-cell Lymphoma: Part of the Spectrum of Cardiac Lymphomas

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.

[1]  I. Gill,et al.  Age-related Epstein-Barr virus-associated lymphoproliferative disorder presenting as renal mass. , 2009, Urology.

[2]  S. de Vos,et al.  Kaposi's sarcoma-associated herpesvirus (KSHV or HHV8) in primary effusion lymphoma: ultrastructural demonstration of herpesvirus in lymphoma cells. , 1996, Blood.

[3]  M. Carrier,et al.  Cardiac lymphoma following mitral valve replacement. , 2005, The Annals of thoracic surgery.

[4]  V. Jha,et al.  Primary cardiac post-transplantation lymphoproliferative disorder—T cell type: a case report and review of the literature , 2006, Journal of Clinical Pathology.

[5]  B. Albat,et al.  Surgical treatment for primary lymphoma of the heart simulating prosthetic mitral valve thrombosis. , 1994, The Journal of thoracic and cardiovascular surgery.

[6]  W. Edwards,et al.  Primary cardiac plasmablastic (diffuse large B-cell) lymphoma mimicking left ventricular aneurysm with mural thrombus. , 2007, Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology.

[7]  T. Terada.  Primary cardiac lymphoma. , 2007, The Tokai journal of experimental and clinical medicine.

[8]  R. Lai,et al.  Indolent mantle cell leukemia: a clinicopathological variant characterized by isolated lymphocytosis, interstitial bone marrow involvement, kappa light chain restriction, and good prognosis , 2011, Haematologica.

[9]  H. Billett,et al.  Primary cardiac lymphoma , 2011, Cancer.

[10]  K. Ohshima,et al.  Senile EBV+ B-Cell Lymphoproliferative Disorders: A Clinicopathologic Study of 22 Patients , 2003, The American journal of surgical pathology.

[11]  M. Sheppard,et al.  Unusual presentation of primary cardiac lymphoma. , 2009, Interactive cardiovascular and thoracic surgery.

[12]  Y. Wakida,et al.  Primary lymphoma of the heart: Case report and literature review , 2004, Pathology international.

[13]  P. Ouillette,et al.  Validation of immunoglobulin gene rearrangement detection by PCR using commercially available BIOMED-2 primers , 2006, Leukemia.

[14]  D. Moodie,et al.  Malignant Primary Cardiac Tumors : The Cleveland Clinic Experience, 1956 to 1986 , 1987 .

[15]  K. Aozasa,et al.  Pyothorax-associated lymphoma: a review of 106 cases. , 2002, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[16]  S. Pileri,et al.  WHO classification of tumours of haematopoietic and lymphoid tissues in 2008: an overview. , 2010, Pathologica.

[17]  H. R. Bergen,et al.  Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens. , 2009, Blood.

[18]  A. Bagatzounis,et al.  Primary Cardiac Lymphoma: Case Report and Brief Review of the Literature , 2009, Echocardiography.

[19]  T. Naoe,et al.  Age-Related EBV-Associated B-Cell Lymphoproliferative Disorders Constitute a Distinct Clinicopathologic Group: A Study of 96 Patients , 2007, Clinical Cancer Research.

[20]  R. Sharpe,et al.  A unique atrial primary cardiac lymphoma mimicking myxoma presenting with embolic stroke: a case report. , 2003, Blood.

[21]  E. Buffolo,et al.  Post-transplant lymphoproliferative disease presenting as a mass in the left ventricle in a heart transplant recipient at long-term follow-up. , 2009, The Journal of Heart and Lung Transplantation.

[22]  L. Staudt,et al.  Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. , 2004, Blood.

[23]  A. Feldman,et al.  Seroma-associated primary anaplastic large-cell lymphoma adjacent to breast implants: an indolent T-cell lymphoproliferative disorder , 2008, Modern Pathology.

[24]  G. Pinkus,et al.  Primary Cardiac Lymphoma: Clinical, Histologic, Immunophenotypic, and Genotypic Features of 5 Cases of a Rare Disorder , 2007, The American journal of surgical pathology.

[25]  A. Feldman,et al.  Epstein-Barr Virus-associated Diffuse Large B-cell Lymphoma Arising on Cardiac Prostheses , 2010, The American journal of surgical pathology.

[26]  I. Khan,et al.  Clinical Perspectives of Primary Cardiac Lymphoma , 2003, Angiology.

[27]  M. Omine.  [HIV-associated lymphoma]. , 1998, Ryoikibetsu shokogun shirizu.

[28]  E. Cesarman,et al.  Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. , 1996, Blood.

[29]  V. Devita,et al.  Heart in malignant lymphoma (Hodgkin's disease, lymphosarcoma, reticulum cell sarcoma and mycosis fungoides). A study of 196 autopsy cases. , 1968, The American journal of cardiology.