A rare case of primary cardiac B cell lymphoma

Abstract

Primary cardiac lymphomas represent an extremely rare entity of extranodal lymphomas and should be distinguished from secondary cardiac involvement of disseminated lymphomas belonging to the non-Hodgkin’s classification of blood cancers. Only 90 cases have been reported in literature. Presentation of cardiac lymphomas on imaging studies may not be unambiguous since they potentially mimic other cardiac neoplasms including myxomas, angiosarcoma or rhadomyomas and therefore require multimodality cardiac imaging, endomyocardial biopsy, excisional intraoperative biopsy and pericardial fluid cytological evaluation to establish final diagnosis.Herein we report the case of a 70 y/o immunocompetent Caucasian female with a rapidly progressing superior vena cava syndrome secondary to a large primary cardiac diffuse large B cell lymphoma (NHL lymphoma) almost completely obstructing the right atrium, right ventricle and affecting both mitral and tricuspid valve. The patient had no clinical evidence of disseminated disease and was successfully treated with extensive debulking during open-heart surgery on cardiopulmonary bypass and 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy (R-CHOP).

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.…”

Pericarditis with anaemia as a herald syndrome in a fatal presentation of cardiac lymphoma

“…Therefore, diagnosis of PCL requires further imaging with transoesophageal echocardiography, cardiac MRI, CT and positron emission tomography scanning, leading to endomyocardial biopsy 9. Only when this histological (or, more rarely, cytological via pericardial fluid) diagnosis is obtained, can the patient be considered for chemotherapy or surgery.…”

Classical nodular sclerosis Hodgkin lymphoma presenting with atypical cardiac involvement

“…Dissemination can occur by lymphatic, bloodstream or contiguous extension [2,17]. Imagistic examination (ultrasonography, CT, PET/CT) has a significant role in establishing the degree of myocardial and pericardic infiltration, extracardiac dissemination and heart function [8].…”

“…Disadvantages of TTE include a potentially restricted field of view and underestimation of tumor extension. Cardiac computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) are further required to fully characterize tumor size and density, myocardial infiltration and metabolic activity [4,8,9].…”