Elsevier

Cardiovascular Pathology

Volume 24, Issue 1, January–February 2015, Pages 60-64
Cardiovascular Pathology

Clinical Case Report
Large B-cell lymphoma arising in cardiac myxoma or intracardiac fibrinous mass: a localized lymphoma usually associated with Epstein–Barr virus?

https://doi.org/10.1016/j.carpath.2014.08.007Get rights and content

Abstract

Primary cardiac neoplasms are rare. However, among them, cardiac myxoma is the most common tumor. In contrast, primary cardiac lymphoma within a cardiac myxoma is extremely rare and might be difficult to diagnose because of non-specific clinical manifestations. We report the case of a previously healthy 52-year-old man who presented with acute onset of transient dysarthria and left hemiplegia. A transthoracic echocardiography showed a 6×2.5-cm solid mass in the left atrium, which was subsequently resected. Histological, immunohistochemical, and molecular analyses revealed an EBV-associated CD30-positive large B-cell lymphoma with anaplastic morphology within a cardiac myxoma and fibrinous material. Staging studies showed no evidence of lymphoma elsewhere. The patient achieved complete remission and is alive 42 months after diagnosis, and did not receive chemotherapy. We discuss the clinical and pathologic features of lymphoma arising in cardiac myxoma or in intra-atrial fibrinoid mass and the potential role of IL-6 in its pathogenesis.

Introduction

Primary cardiac neoplasms are rare. About 80%–90% of primary cardiac tumors are benign [1], [2], and of these the most common are cardiac myxomas, which usually arise in the left atrium [1], [2], [3]. Most patients present with one or more symptoms of the classic triad of embolic events, intracardiac flow obstruction and constitutional symptoms [2], [3], [4], and about 10% of patients are asymptomatic [3].

Primary cardiac lymphoma is a rare neoplasm, accounting for 2% of all primary cardiac tumors, and affects mainly the right chambers of the heart [5]. During the last decade, isolated cases of lymphoproliferative neoplasms within a cardiac myxoma [6], [7], [8], [9], [10], [11], [12], or intracardiac fibrinoid masses [13], [14], [15], [16], [17], [18], [19], occasionally have been reported in the medical literature; most of these reports show an association with Epstein–Barr virus (EBV) infection and show a local production of inflammatory cytokines.

We report the clinical, histologic, phenotypic, and molecular findings of a patient with large B-cell lymphoma associated with EBV infection, discovered incidentally within a myxomatous and fibrinoid cardiac mass.

Section snippets

Case presentation

A 52-year-old man without any significant past medical history was admitted to our hospital with acute onset of transient dysarthria and left hemiplegia, after a soccer game. On cardiac examination, he had regular rate and rhythm. No murmurs or gallops were audible on auscultation. Laboratory tests showed increased C-reactive protein (4.3 mg/dl) and lactate dehydrogenase levels (538 U/l). Complete blood counts and metabolic panel were normal. Computed tomography (CT) scan of the head suggested

Discussion

We report an immunocompetent patient who presented with an incidental CD30-positive diffuse large B-cell lymphoma (DLBCL) arising within a left heart atrium myxomatous and fibrinoid mass. The patient had surgical removal of the mass, and did not need chemotherapy; patient has no evidence of disease 42 months after diagnosis. The association of the DLBCL with EBV type III latency without an associated underlying immunosuppression of the patient, suggests that unknown mechanisms of local

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  • Cited by (0)

    Funding: Authors disclose no funding sources.

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