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Case Reports
. 2009 Jun;25(6):e210-2.
doi: 10.1016/s0828-282x(09)70110-2.

Primary cardiac diffuse large B cell lymphoma presenting with superior vena cava syndrome

Amer Johri  1 , Tara Baetz, Phillip A Isotalo, Robert L Nolan, Anthony J Sanfilippo, Glorianne Ropchan
Affiliations
Case Reports

Primary cardiac diffuse large B cell lymphoma presenting with superior vena cava syndrome

Amer Johri et al. Can J Cardiol. 2009 Jun.

Abstract
in English, French

Primary cardiac lymphomas are rare extranodal lymphomas that should be distinguished from secondary cardiac involvement by disseminated non-Hodgkin's lymphoma. Cardiac lymphomas often mimic other cardiac neoplasms, including myxomas and angiosarcomas, and often require multimodality cardiac imaging, in combination with endomyocardial biopsy, excisional biopsy or pericardial fluid cytology, to establish a definitive diagnosis. A 60-year-old immunocompetent man who presented with superior vena cava syndrome secondary to a right atrial, primary cardiac diffuse large B cell lymphoma (non-Hodgkin's lymphoma) is described in the present article. The patient had no clinical evidence of disseminated lymphoma and was successfully treated with prompt surgical excision of his atrial mass, followed by anthracycline-based chemotherapy. The patient required multi-modality cardiac imaging to accurately identify and plan surgical excision of his cardiac lymphoma. The therapeutic management and clinical and radio-logical features of primary cardiac lymphoma are reviewed.

Les lymphomes cardiaques primaires sont des lymphomes extranodaux rares qu’il faudrait distinguer d’une atteinte cardiaque secondaire par lymphome non hodgkinien disséminé. Les lymphomes cardiaques imitent souvent d’autres néoplasmes cardiaques, y compris les myxomes et les angiosarcomes, et exigent souvent une imagerie cardiaque multimode, conjointement avec une biopsie endomyocardique, une biopsie-exérèse ou une cytologie du liquide péricardique, afin de poser un diagnostic définitif. Un homme immunocompétent de 60 ans qui a consulté en raison d’un syndrome de compression de la veine cave supérieure secondaire à un lymphome cardiaque primaire diffus à grandes cellules bêta (lymphome non hodgkinien) est décrit dans le présent article. Le patient ne présentait aucune constatation clinique de lymphome disséminé et a reçu un traitement concluant par excision chirurgicale rapide de la masse auriculaire, suivi d’une chimiothérapie à l’anthracycline. Il a dû subir une imagerie cardiaque multimode pour repérer avec précision et planifier l’excision chirurgicale du lymphome cardiaque. L’auteur examine la prise en charge thérapeutique et les caractéristiques cliniques et radiologiques du lymphome cardiaque primaire.

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Figures

Figure 1)
Figure 1)
Transthoracic echocardiogram with subcostal views showing a homogenous, immobile, soft tissue density measuring 3.5 cm × 4.5 cm within the right atrium. The mass appears to partially occlude superior vena cava flow but does not appear to extend into either vena cavae
Figure 2)
Figure 2)
Computed tomography scan demonstrating a 4.9 cm right atrial mass (arrows) that nearly fills the atrial chamber. A thin rim of contrast enhancement surrounding the right atrial mass is also seen. There is no evidence of mediastinal lymphadenopathy or pulmonary/skeletal disease
Figure 3)
Figure 3)
A Future right atriotomy site demonstrating surface sclerosis. B Incision into the right atrium, 1 cm lateral to the atrioventricular junction, and the course of the right coronary artery revealed an atrial mass that immediately bulged out through the atriotomy. The mass had an anteromedial attachment and did not invade the vena cavae or other cardiac structures
Figure 4)
Figure 4)
Bovine pericardial patch anastamosed to the extensive resection margin
Figure 5)
Figure 5)
Excised primary cardiac lymphoma demonstrating a homogeneous ‘fish-flesh’ gross appearance that is often typical of lymphoproliferative disorders. Histopathological examination confirmed the tumour to be a diffuse, large B cell lymphoma with plasmacytoid differentiation
See this image and copyright information in PMC

References

    1. Burke A, Virmani R. Atlas of Tumor Pathology (Third series, fascicle 16): Tumors of the Heart and Great Vessels. Washington: Armed Forces Institute of Pathology; 1996. pp. 171–9.
    1. Ceresoli GL, Ferreri AJM, Bucci E, Ripa C, Ponzoni M, Villa E. Primary cardiac lymphoma in immunocompetent patients: Diagnostic and therapeutic management. Cancer. 1997;80:1497–506. - PubMed
    1. Nascimento AF, Winters GL, Pinkus GS. Primary cardiac lymphoma: Clinical, histologic, immunophenotypic, and genotypic features of 5 cases of a rare disorder. Am J Surg Pathol. 2007;31:1344–50. - PubMed
    1. Schell AJ, Xu Y, Baetz T, et al. Primary cardiac lymphoma: Molecular cytogenetic characterization of a rare entity. Cardiovasc Pathol. 2009;18:92–9. - PubMed
    1. Rolla G, Galligaris-Cappio F, Burke AP. Cardiac lymphomas. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. World Health Organization Classification of Tumours Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. 1st edn. Lyon: IARC Press; 2004. pp. 282–3.

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