A rare case of primary cardiac B cell lymphoma

Andreas Habertheuer,Alfred Kocher,Marek Ehrlich,Claus Rath,Dominik Wiedemann,Bruno Mora

doi:10.1186/1749-8090-9-14

Andreas Habertheuer, Alfred Kocher + Show 4 more

Open Access

https://doi.org/10.1186/1749-8090-9-14

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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).

Highlights

Primary malignant cardiac neoplasms are extremely rare and among those primary lymphomas constitute only a minor entity
The operation was performed with inferior vena cava and anonymous vein cannulation due to the fact that the superior vena cava (SVC) was obstructed and a left ventricular vent via the right superior pulmonary vein on cardiopulmonary bypass
Primary cardiac lymphomas must be distinguished from other primary malignant tumors of the heart such as angiosarcomas and from the more common benign cardiac tumors such as myxomas and lipomas

Summary

Background

Primary malignant cardiac neoplasms are extremely rare and among those primary lymphomas constitute only a minor entity. The operation was performed with inferior vena cava and anonymous vein cannulation due to the fact that the SVC was obstructed and a left ventricular vent via the right superior pulmonary vein on cardiopulmonary bypass. Myocardial protection was achieved with antegrade aortic cardioplegia only Both the right atrial and right ventricular walls and cavitites consisted of dense, fibrotic composition and tumor masses were palpable along the right side of the heart. Surgical debulking was feasible as the entire right side of the heart appeared to consist only of tumor mass. Despite the highly malignant nature of the lymphoma the regimen was initiated only after a time span of 2 weeks after surgery due to major concerns of potential ventricular rupture as the neoplasm was diffusely infiltrating cardiac structures including the entire free wall of the right ventricle. Post-surgical adhesions between the heart and the pericardial sac were thought to prevent major bleedings beyond this 2-week period

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