Elsevier

Cardiovascular Pathology

Volume 31, November–December 2017, Pages 54-56
Cardiovascular Pathology

Clinical Case Report
Primary cardiac diffuse large B-cell lymphoma with concurrent high MYC and BCL2 expression in an immunocompetent Chinese elderly woman

https://doi.org/10.1016/j.carpath.2017.07.006Get rights and content

Highlights

  • Primary cardiac diffuse large B-cell lymphoma

  • With MYC and BCL2 protein co-expression accompanying with MYC gene rearrangement.

  • Data are very limited regarding primary cardiac lymphoma with DEL or DHL characteristics.

Abstract

Primary cardiac lymphoma is a rare type of extranodal lymphoma that involves only heart and/or pericardium. It is usually observed in immunodeficient people. However, immunocompetent patients may also suffer from this disease. Most primary cardiac lymphomas are of B-cell lineage, and they usually present as diffuse large B-cell lymphoma (DLBCL). Diffuse large B-cell lymphoma with concurrently high MYC and BCL2 expression, which is named as double-expressor lymphoma (DEL), is a rare subtype of DLBCL. Here we report a rare case of a primary cardiac DEL in an immunocompetent 65-year old Chinese woman. Echocardiography and magnetic resonance imaging revealed a mass of 6.6 cm×5.6 cm in the right atrium. No tumor formations were observed in other organs. Histopathologic examination showed that the cardiac tumor was diffuse large B-cell lymphoma, non-germinal center B-cell type by Hans algorithm. The tumor cells showed high MYC and BCL2 protein expression by immunohistochemistry, with high proliferative index.

Introduction

Primary cardiac lymphoma (PCL) has been classified as an extranodal lymphoma, involving only heart and/or pericardium in the latest WHO classification of tumors of the lung, pleura, thymus and heart. Primary cardiac lymphomas are rare tumors that account for <2% of all primary cardiac neoplasms [1]. Here, we report a very rare case of primary cardiac diffuse large B-cell lymphoma of non-germinal center B-cell type with MYC and BCL2 protein co-expression in an immunocompetent patient.

Section snippets

Case report

A 65-year old woman, who had a 10-year history of hypertension, presented with face swelling, chest discomfort and asthma after physical activity for 4 months. Electrocardiogram showed that the patient has sick sinus syndrome. Echocardiography revealed a low echo mass in the right atrium suggesting the possibility of cardiac myxoma. Magnetic Resonance Imaging (MRI) revealed a soft tissue mass measuring 6.6 cm×5.7 cm in the right atrium which partly extended to the superior vena cava, inferior

Materials and methods

The surgically resected specimen was fixed in 10% neutral buffered formalin and embedded in paraffin. Hematoxylin–eosin staining and immunohistochemical analysis were performed on four μM thick paraffin-embedded sections. The following primary antibodies were used: CD20 (clone L26; Dako, Carpentaria, CA, USA), CD3ε (polyclonal), CD10 (clone 56C6; Dako), BCL6 (clone PG-B6p; Dako), MUM1 (cloneMUM1p; Dako), BCL2 (clone 124; Dako), MYC (clone Y69; Epitomics, Burlingame, CA, USA), Ki-67 (clone

Histology

Microscopical examination revealed that the tumor was composed of diffusely infiltrating large lymphoid cells with vesicular chromatin and round or oval nuclei, which show high mitotic activity (Fig. 2A).

Immunohistochemistry

Immunohistochemical staining showed that the tumor cells were positive for CD20, CD45, CD5, MUM-1, and negative for CD3, CD30, BCL6, CD10, Cyclin D1, NF-κB. Approximately 50% and 60% of tumor cells expressed BCL2 and MYC, respectively. Ki-67 index was 85% (Fig. 2B–E).

In situ hybridization

Tumor cells did not show

Discussion

Nearly 70–80% of primary heart tumors are benign. Most malignant cardiac tumors are metastatic tumors and only a few are primary cardiac neoplasms. Cardiac myxoma is the most common primary cardiac neoplasm which usually presents in the left atrium. On the other hand, primary cardiac lymphoma (PCL) is a relatively rare malignancy, and it accounts for less than 2% of primary cardiac neoplasms [1]. Primary cardiac lymphoma incidence is slightly higher in male patients, and the elderly are more

Conflict of interest

Disclosure statement

All authors disclosed no financial relationships relevant to this publication.

References (18)

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Funding: This study was supported by a grant founded by the Health and Family Planning Commission of Sichuan Province (no:16PJ337), and a grant of application basic research project founded by the Science and Technology Department of Sichuan Province (no:2017JY0266).

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