A Malignant Lymphoma Growing Inside a Cardiac Mixoma: A Case Report

Pirola, Sergio; Fiori, Stefano; Maffini, Fausto; Mostardini, Giulia; Mastroiacovo, Giorgio; Polvani, Gianluca

doi:10.21470/1678-9741-2021-0081

Sergio Pirola Correspondence Address: Sergio Pirola, https://orcid.org/0000-0002-4573-8346 IRCCS Centro Cardiologico Monzino, street Parea 4, Milan, Italy - Zip code: 20138, E-mail: sergio.pirola@ccfm.it

Department of Cardiac Surgery, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

* These authors equally contributed to this manuscript

https://orcid.org/0000-0002-4573-8346

Stefano Fiori

Division of Diagnostic Haematopathology, European Institute of Oncology, IRCCS, Milan, Italy.

Fausto Maffini

Division of Pathology, European Institute of Oncology IRCCS, Milan, Italy.

Giulia Mostardini

Department of Cardiac Surgery, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

Giorgio Mastroiacovo

Department of Cardiac Surgery, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

Department of Cardiovascular Sciences and Community Health, University of Milan, Italy.

* These authors equally contributed to this manuscript

Gianluca Polvani

Department of Cardiac Surgery, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

Department of Cardiovascular Sciences and Community Health, University of Milan, Italy.

*
These authors equally contributed to this manuscript

This study was carried out at the Department of Cardiac Surgery, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

Correspondence Address: Sergio Pirola, https://orcid.org/0000-0002-4573-8346 IRCCS Centro Cardiologico Monzino, street Parea 4, Milan, Italy - Zip code: 20138, E-mail: sergio.pirola@ccfm.it

No conflict of interest.

Figures (3)
Tables (3)

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Fig. 1
2D transthorachic echocardiography images and 3D reconstruction of the left atrial mass.

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Fig. 2
CT-based 3D virtual reconstruction of the mass performed using MIMICS software showing the extremely irregular borders and a narrow implantation stalk in the fossa ovalis.

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Fig. 3
A, B, C: Morphological features of the lesion. Whole-mount section of myxoma, with villous surface and a superficial fibrinous cap (arrows) containing lymphoid aggregates (A, haematoxilin-eosin). The myxoma consists of a translucent loose matrix containing scattered stellate cells (asterisk). The lymphoid cells embedded in fibrinous material (arrowhead) are large-sized and atypical (B, C, inset, haematoxilin-eosin). D, E, F: Immunophenotypic features of fibrin-associated diffuse large B-cell lymphoma. The atypical lymphoid cells have a B-cell phenotype, being positive for CD20 (A) and PAX5 (B), are diffusely positive for EBV/EBER (C) and have a high Ki67 proliferation index (D), in contrast to the myxoma cells (D, upper half), which are almost completely

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Abbreviations, acronyms & symbols CCS = Canadian Cardiovascular Society CT = Computed tomography DLBCL-NOS = Large B-cell lymphoma not otherwise specified EBV = Epstein-Barr virus EBER = Epstein-Barr virus-encoded small RNAs FA-DLBCL = Fibrin-associated diffuse large B-cell lymphoma LMP1 = Latent membrane protein 1 NYHA = New York Heart Association

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Table 1
Available literature data regarding cases of myxomas in which B-cell lymphomas were identified.

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Authors' roles & responsibilities SP Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work; drafting the work or revising it critically for important intellectual content; final approval of the version to be published SF Histological analysis of the surgical specimen, curation of the anatomopathological part of the paper FM Histological analysis of the surgical specimen, curation of the anatomopathological part of the paper. GM Radiological image processing and reconstruction with special editing software (MIMICS, Materialise) GM Collaboration in writing the paper and proofreading it, as well as editing the literature review GP Coordination and supervision of the entire work of the team of researchers and final endorsement to the publication of the paper

Abbreviations, acronyms & symbols
CCS	= Canadian Cardiovascular Society
CT	= Computed tomography
DLBCL-NOS	= Large B-cell lymphoma not otherwise specified
EBV	= Epstein-Barr virus
EBER	= Epstein-Barr virus-encoded small RNAs
FA-DLBCL	= Fibrin-associated diffuse large B-cell lymphoma
LMP1	= Latent membrane protein 1
NYHA	= New York Heart Association

First author	Location and size	Symptoms	Lymphoma	EBV type	Gross analysis	Follow-up
Bagwan[¹⁷17 Bagwan IN, Desai S, Wotherspoon A, Sheppard MN. Unusual presentation of primary cardiac lymphoma. Interact Cardiovasc Thorac Surg. 2009;9(1):127-9. doi:10.1510/icvts.2009.204628. https://doi.org/10.1510/icvts.2009.20462... ]	Left atrium (4 × 2 cm)	Multiple strokes	Diffuse large B-cell lymphoma	Not available	Gelatinous, grape-like, friable	Not available
Dimitrova[¹⁸18 Dimitrova KR, Hoffman DM, Geller CM, Thiagarjah P, Master J, Berger M, et al. Malignant B-cell lymphoma arising in a large, left atrial myxoma. Ann Thorac Surg. 2010;89(2):626-9. doi:10.1016/j.athoracsur.2009.06.096. https://doi.org/10.1016/j.athoracsur.200... ]	Left atrium (7.5 × 4.5 cm)	Chest pain	Diffuse large B-cell lymphoma	Not available	Partially fimbriated surface, gelatinous and necrotic parts	Not available
Loong[¹¹11 Loong F, Chan AC, Ho BC, Chau YP, Lee HY, Cheuk W, et al. Diffuse large B-cell lymphoma associated with chronic inflammation as an incidental finding and new clinical scenarios. Mod Pathol. 2010;23(4):493-501. doi:10.1038/modpathol.2009.168. https://doi.org/10.1038/modpathol.2009.1... ]	Left atrium (6.5 × 4 cm)	Cardiogenic shock, ischemic stroke	Diffuse large B-cell lymphoma	Type III latency (EBER+, LMP1+)	Not described	Patient deceased for chemotherapy complications (neutropenia + pneumonia) at 5 months
Svec[¹²12 Svec A, Rangaiah M, Giles M, Jaksa R, McAulay KA. EBV+ diffuse large B-cell lymphoma arising within atrial myxoma. An example of a distinct primary cardiac EBV+ DLBCL of immunocompetent patients. Pathol Res Pract. 2012;208(3):172-6.]	Left atrium (3.7 × 1.5 cm)	Ischemic stroke	Diffuse large B-cell lymphoma	Type III latency (EBER +, LMP1+)	Not described	No evidence of disease at 7 months
Bartoloni[¹³13 Bartoloni G, Pucci A, Giorlandino A, Berretta M, Mignosa C, Italia F, et al. Incidental Epstein-Barr virus associated atypical lymphoid proliferation arising in a left atrial myxoma: a case of long survival without any postsurgical treatment and review of the literature. Cardiovasc Pathol. 2013;22(3):e5-10. doi:10.1016/j.carpath.2012.08.002. https://doi.org/10.1016/j.carpath.2012.0... ]	Left atrium (5.5 × 4.5 cm)	Fever and progressive fatigue	Atypical lymphoid B-cell proliferation	Type II latency (EBER +, LMP1+)	Glistening, villous and gelatinous surface	No evidence of disease at 72 months
Tapan[¹⁵15 Tapan U, Pestana JB, Lee JC, Lerner A. Epstein-Barr virus-associated diffuse large B-cell lymphoma arising in atrial myxoma: a proposal for a modified therapeutic approach. Leuk Lymphoma. 2015;56(2):505-7. doi:10.3109/10428194.2014.919632. https://doi.org/10.3109/10428194.2014.91... ]	Left atrium (4.2 × 3.6 cm)	Palpitations	Diffuse large B-cell lymphoma	Type III latency (EBER +, LMP1+)	Not described	No evidence of disease at 12 months
Aguilar[¹⁴14 Aguilar C, Beltran B, Quiñones P, Carbajal T, Vilcapaza J, Yabar A, et al. Large B-cell lymphoma arising in cardiac myxoma or intracardiac fibrinous mass: a localized lymphoma usually associated with Epstein-Barr virus? Cardiovasc Pathol. 2015;24(1):60-4. doi:10.1016/j.carpath.2014.08.007. https://doi.org/10.1016/j.carpath.2014.0... ]	Left atrium (6 × 2.5 cm)	Transient ischemic attack	Diffuse large B-cell lymphoma	Type III latency (EBER +, LMP1+)	Not described	No evidence of disease at 42 months
Pineda[³3 Pineda AM, Mihos CG, Nascimento FO, Santana O, Lamelas J, Beohar N. Coronary embolization from a left atrial myxoma containing malignant lymphoma cells. Tex Heart Inst J. 2015;42(6):565-8. doi:10.14503/THIJ-14-4619. https://doi.org/10.14503/THIJ-14-4619... ]	Left atrium (6.5 × 3 cm)	Coronary embolization	Diffuse large B-cell lymphoma	Not available	Reddish, gelatinous	Not available
Park[²⁰20 Park CK, Cho YA, Kim M, Shim HS. Malignant lymphoma arising in cardiac myxoma, presenting with peripheral arterial emboli. Cardiovasc Pathol. 2018;32:26-9. doi:10.1016/j.carpath.2017.10.001. https://doi.org/10.1016/j.carpath.2017.1... ]	Left atrium (6 × 3.2 cm)	Peripheral arterial embolization	Diffuse large B-cell lymphoma	Type III latency, EBER +	Irregular surface, friable	Not available
Boyer[⁹9 Boyer DF, McKelvie PA, de Leval L, Edlefsen KL, Ko YH, Aberman ZA, et al. Fibrin-associated EBV-positive large B-cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-cell lymphoma associated with chronic inflammation. Am J Surg Pathol. 2017;41(3):299-312. doi:10.1097/PAS.0000000000000775. https://doi.org/10.1097/PAS.000000000000... ]	Left atrium (? cm)	Syncope	Diffuse large B-cell lymphoma	EBER+	Not available	No evidence of disease at 130 months
Boyer[⁹9 Boyer DF, McKelvie PA, de Leval L, Edlefsen KL, Ko YH, Aberman ZA, et al. Fibrin-associated EBV-positive large B-cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-cell lymphoma associated with chronic inflammation. Am J Surg Pathol. 2017;41(3):299-312. doi:10.1097/PAS.0000000000000775. https://doi.org/10.1097/PAS.000000000000... ]	Left atrium (? cm)	Syncope, cough, dyspnoea	Diffuse large B-cell lymphoma	EBER+, LMP1+	Reddish, gelatinous	Patient died at 2 months for cardiac cause
Boyer[⁹9 Boyer DF, McKelvie PA, de Leval L, Edlefsen KL, Ko YH, Aberman ZA, et al. Fibrin-associated EBV-positive large B-cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-cell lymphoma associated with chronic inflammation. Am J Surg Pathol. 2017;41(3):299-312. doi:10.1097/PAS.0000000000000775. https://doi.org/10.1097/PAS.000000000000... ]	Left atrium (? cm)	Dyspnoea, respiratory failure	Diffuse large B-cell lymphoma	EBER+, LMP1+	Not available	Recurrent FA-DLBCL at mitral valve after 25 months. Patient died at 26 months (embolic stroke).
Yan[¹⁰10 Yan J, Luo D, Zhang F, He J, Yao S, Luo X, et al. Diffuse large B cell lymphoma associated with chronic inflammation arising within atrial myxoma: aggressive histological features but indolent clinical behaviour. Histopathology. 2017;71(6):951-9. doi:10.1111/his.13336. https://doi.org/10.1111/his.13336... ]	Left atrium (? cm)	Congestive heart failure	Diffuse large B-cell lymphoma	Type III latency, EBER+, LMP1+	Myxoid appearance with an abundant fibrinous or mucinous background in the centre	No evidence of disease at 7 months
Yan[¹⁰10 Yan J, Luo D, Zhang F, He J, Yao S, Luo X, et al. Diffuse large B cell lymphoma associated with chronic inflammation arising within atrial myxoma: aggressive histological features but indolent clinical behaviour. Histopathology. 2017;71(6):951-9. doi:10.1111/his.13336. https://doi.org/10.1111/his.13336... ]	Left atrium (? cm)	Congestive heart failure	Diffuse large B-cell lymphoma	Type III latency, EBER+, LMP1+	Myxoid appearance with an abundant fibrinous or mucinous background in the centre	No evidence of disease at 84 months
Yan[¹⁰10 Yan J, Luo D, Zhang F, He J, Yao S, Luo X, et al. Diffuse large B cell lymphoma associated with chronic inflammation arising within atrial myxoma: aggressive histological features but indolent clinical behaviour. Histopathology. 2017;71(6):951-9. doi:10.1111/his.13336. https://doi.org/10.1111/his.13336... ]	Left atrium (? cm)	Congestive heart failure	Diffuse large B-cell lymphoma	Type III latency, EBER+, LMP1+	Myxoid appearance with an abundant fibrinous or mucinous background in the centre	No evidence of disease at 3 months
Yan[¹⁰10 Yan J, Luo D, Zhang F, He J, Yao S, Luo X, et al. Diffuse large B cell lymphoma associated with chronic inflammation arising within atrial myxoma: aggressive histological features but indolent clinical behaviour. Histopathology. 2017;71(6):951-9. doi:10.1111/his.13336. https://doi.org/10.1111/his.13336... ]	Left atrium (? cm)	Congestive heart failure	Diffuse large B-cell lymphoma	Type III latency, EBER+, LMP1+	Myxoid appearance with an abundant fibrinous or mucinous background in the centre	No evidence of disease at 120 months
Present case	Left atrium (3.6 × 3 cm)	Asthenia, dyspnoea and dizziness	Diffuse large B-cell lymphoma	EBER+, LMP1+	Gelatinous, grape-like, pale pink, semi-transparent, extremely friable	No evidence of disease at 4 months

Authors' roles & responsibilities
SP	Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work; drafting the work or revising it critically for important intellectual content; final approval of the version to be published
SF	Histological analysis of the surgical specimen, curation of the anatomopathological part of the paper
FM	Histological analysis of the surgical specimen, curation of the anatomopathological part of the paper.
GM	Radiological image processing and reconstruction with special editing software (MIMICS, Materialise)
GM	Collaboration in writing the paper and proofreading it, as well as editing the literature review
GP	Coordination and supervision of the entire work of the team of researchers and final endorsement to the publication of the paper

[1] *
These authors equally contributed to this manuscript

Brasil

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A Malignant Lymphoma Growing Inside a Cardiac Mixoma: A Case Report

Abstract

Introduction:

Conclusion: