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A Malignant Squeeze: A Rare Cause of Cardiac Tamponade.

1. Introduction

Primary cardiac lymphoma (PCL) is a rare condition that is described as a lymphoma localized to the heart or pericardium with no extracardiac involvement. Although cardiac involvement is seen in about 10-20% of non-Hodgkin's lymphomas, PCL is extremely rare. It comprises merely 0.5% of all lymphomas and 1.3-2% of cardiac malignancies [1, 2]. Clinical presentation typically correlates with the region of the heart involved. It is often a clinical emergency as these patients commonly present with heart failure, cardiac tamponade, or arrhythmia [3]. Herein, we describe a rare case of PCL causing cardiac tamponade in an elderly patient.

2. Case Description

An 85-year-old female with past medical history of recurrent deep venous thrombosis, pulmonary embolism on anticoagulation with a vena cava filter in place, rheumatoid arthritis, hypertension, hyperlipidemia, hypothyroidism, and type 2 diabetes mellitus presented to the emergency department with complaint of exertional dyspnea and chest pain. She denied fever, chills, or lower extremity edema and had no history of malignancy, weight loss, or night sweats. Initial vitals revealed BP of 86/62 mmHg that decreased to 79/60 mmHg with inspiration. Initial pulse was 95 bpm, and respiratory rate was 20. White blood cell count was mildly elevated at 11.5, troponins were normal, and electrocardiogram was unremarkable. D-dimer was elevated at 1290. Patient was sent for CT scan to evaluate for pulmonary embolism, and a moderate pericardial effusion was found (Figure 1). Subsequently, an echocardiogram was done to further delineate the effusion which revealed a large circumferential effusion with mild respiratory variation concerning for impending cardiac tamponade (Figure 2). She was admitted to the intensive care unit and underwent emergent pericardiocentesis. Flow cytometry of the pericardial fluid revealed a population of monoclonal B-cells with significant large cell component (Figure 3). The overall morphologic and immunophenotypic features were consistent with high-grade B-cell lymphoma with t(8; 144 (Figure 4). Bone marrow biopsy demonstrated monotypic B-cells compatible with the diagnosis of large B-cell lymphoma. The patient was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) with an excellent initial response. She was transferred out of the ICU within days and discharged home for outpatient follow-up.

3. Discussion

Primary cardiac tumors are known classically to be rare and benign. Cardiac myxoma is the most frequently reported primary cardiac tumor, but hematologic disease, in rare cases, has been known to orginate in the heart [4]. Lymphomas compromise only 0.5-2% of primary cardiac tumors and occur most often in immunocompromised patients, such as those with human immunodeficiency virus (HIV) [5]. The frequencies of other types of non-Hodgkin's lymphoma are detailed in Figure 5 with diffuse large B-cell lymphoma comprising the majority at 30% [6]. PCL does occur in immunocompetent hosts although infrequently, as it did in our patient. In these immunocompetent patients, studies have shown that 80% of PCL are of diffuse B-cell lymphoma [7]. The location of cardiac involvement for PCL is often the right atrium and right ventricle. Clinical presentation can vary depending on the site of involvement of the heart. Most often, an extensive multidisciplinary workup is employed to come up with the correct diagnosis. Imaging modalities such as transthoracic and transesophageal echocardiography, computed tomography (as done in our patient), and magnetic resonance imaging are all utilized in diagnosing this entity. Confirmation of the diagnosis is through cytology of biopsy or pericardial fluid. The combination of chemotherapy and radiation is the treatment of choice. Treatment for B-cell lymphoma is typically an anthracycline-based, such as given in our patient. Our patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Survival has been demonstrated in some cases up to 5 years with treatment but is less than a month without it [7].

Although PCL is rare, the pathophysiology is classic and detection through a detailed physical exam and directed workup diagnosis can be promptly made, even when presenting as tamponade as did our patient. Patients presenting with signs and symptoms of new-onset acute decompensated heart failure can potentially have this diagnosis. Immediate detection is essential to avoid fatal complications, which include cardiac tamponade, ischemia, and fatal arrhythmia. To our knowledge, only 13 cases have described PCL presenting with tamponade physiology and are highlighted in Table 1. Parato et al. describe a case of a younger patient who presented with cardiac tamponade due to PCL. The patient was treated with management of cardiac tamponade, surgical excision of the mass, and chemotherapy. Complete remission was obtained after six months of chemotherapy [8]. Although there was a large age difference, our patient also responded to chemotherapy following medical stabilization. Interestingly, Frikha et al. describe a case of an elderly immunocompromised patient presenting with cardiac tamponade along with paroxysmal third-degree atrioventricular (AV) block. They hypothesized that electrical compromise occurred via extension of the malignancy into the interatrial septum and the nodal tissue [9]. Similarly, Gowda and Khan and Houchaymi et al. describe a case of an elderly immunocompetent male who was found to have pericardial tamponade along with third-degree AV block. However, pericardial fluid analysis did not reveal the diagnosis until a transvenous biopsy of the cardiac tumor revealed non-Hodgkin large B-cell lymphoma [7, 10]. Gowda and Khan and Chiba et al. report a similar case of a middle-aged male with cardiac tamponade and AV block who was found on right atrial tumor biopsy to have a diffuse large B-cell lymphoma. The patient underwent chemotherapy and required permanent pacemaker placement [7, 11]. On our extensive literature search, we identified one case report in which the cardiac tamponade was not caused by the lymphoma itself, but rather due to the malignant right atrial rupturing causing tamponade [12]. Further cases are described in Table 1.

The etiology of PCL is still unknown, and molecular characterization has yet to be studied leaving a great deal of research to be done to gain a better understanding of this rare disease process. Nevertheless, although extremely rare, PCL can present with severe cardiac dysfunction due to tamponade physiology or impending tamponade physiology and heart failure. Clinical suspicion must remain high, and diagnostic studies of pericardial fluid can help prevent delay in management of an aggressive malignancy.

Conflicts of Interest

The authors declare that there is no conflict of interest.


[1] H. Ikeda, S. Nakamura, H. Nishimaki et al., "Primary lymphoma of the heart: case report and literature review," Pathology International, vol. 54, no. 3, pp. 187-195, 2004.

[2] G. L. Ceresoli, A. J. M. Ferreri, E. Bucci, C. Ripa, M. Ponzoni, and E. Villa, "Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management," Cancer, vol. 80, no. 8, pp. 1497-1506, 1997.

[3] C. S. Chim, A. C. L. Chan, Y. L. Kwong, and R. Liang, "Primary cardiac lymphoma," American Journal of Hematology, vol. 54, no. 1, pp. 79-83, 1997.

[4] J. G. Wang, H. Liu, W. J. Yu, Y. J. Li, and F. J. Xin, "Primary cardiac neoplasms: a clinicopathologic analysis of 81 cases," Zhonghua Bing Li Xue Za Zhi, vol. 41, no. 12, pp. 808-12, 2012.

[5] M. Duong, C. Dubois, M. Buisson et al., "Non-Hodgkin's lymphoma of the heart in patients infected with human immunodeficiency virus," Clinical Cardiology, vol. 20, no. 5, pp. 497-502, 1997.

[6] American Society of Clinical Oncology (ASCO), "Lymphoma non-Hodgkin: subtypes," 2016, cancer-types/lymphoma-non-hodgkin/subtypes.

[7] R. M. Gowda and I. A. Khan, "Clinical perspectives of primary cardiac lymphoma," Angiology, vol. 54, no. 5, pp. 599-604, 2003.

[8] V. M. Parato, F. Muscente, and M. Scarano, "Primary cardiac lymphoma: a case report," Giornale Italiano di Cardiologia, vol. 18, no. 1,pp. 11-13, 2017.

[9] Z. Frikha, L. Abid, D. Abid et al., "Cardiac tamponade and paroxysmal third-degree atrioventricular block revealing a primary cardiac non-Hodgkin large B-cell lymphoma of the right ventricle: a case report," Journal of Medical Case Reports, vol. 5, no. 1, p. 433, 2011.

[10] Z. Houchaymi, S. Helou, and J. Ballout, "Pericardial tamponade and third-degree atrioventricular block revealing a primary cardiac lymphoma," La Revue de Medecine Interne, vol. 31, no. 11, pp. e4-e6, 2010.

[11] Y. Chiba, K. Oka, H. Saito, R. Nagayama, M. Murata, and N. Mori, "Primary cardiac B-cell lymphoma presented as heart tamponade and atrioventricular block: a case report," Acta Cytologica, vol. 54, no. 1, pp. 79-81, 2010.

[12] A. Menotti, F. Imperadore, G. Pelosi, and M. Disertori, "Heart rupture at the right atrial level as the first manifestation of malignant lymphoma," Cardiologia, vol. 41, no. 1, pp. 65-67, 1996.

Elisa Quiroz, (1) Adam Hafeez, (1) Ramy Mando, (1) Zhou Yu, (1) and Feroze Momin (2)

(1) Department of Internal Medicine, Beaumont Health, Royal Oak, MI, USA

(2) Department of Hematology and Oncology, Beaumont Health, Royal Oak, MI, USA

Correspondence should be addressed to Ramy Mando;

Received 25 June 2018; Accepted 4 September 2018; Published 23 September 2018

Academic Editor: Jeanine M. Buchanich

Caption: Figure 1: Computed tomography of the chest demonstrating pericardial effusion.

Caption: Figure 2: Echocardiogram demonstrating a large circumferential effusion (outlined) concerning for impending tamponade.

Caption: Figure 3: Histopathology remarkable for monoclonal B-cells with significant large cell components.

Caption: Figure 4: Fluorescence in situ hybridization demonstrating t(8; 14). This translocation is between IGH on chromasome 8 and MYC on chromosome 14.
Table 1: Reports of primary cardiac lymphoma causing
cardiac tamponade.

Article (language,           Description             Highlights

Parato VM, Muscente          35-year-old
F, Scarano M. Primary     immunocompromised
cardiac lymphoma: a       patient presented
case report. G Ital        with signs and
Cardiol (Rome). 2017         symptoms of
Jan; 18(1): 11-13            tamponade.
(Italian, 28287210)       Echocardiography
                         revealed lateral AV
                           mass with large
                        pericardial effusion.
                        Patient had surgical
                          excision of mass
                             followed by
                          chemotherapy that
                          induced complete
                           remission at 6

Tzachanis D, Dewar R,    44-year-old female        Primary Burkitt
Luptakova K, Chang         presented with             lymphoma
JD, et al. Primary       abdominal bloating
cardiac Burkitt         who was found to have
lymphoma presenting      a large pericardial
with abdominal pain         effusion with
(English, 25431699)     tamponade physiology.
                          Pericardial fluid
                           studies led to
                        diagnosis of cardiac
                          Burkitt lymphoma.

Frikha Z, Abid L,            64-year-old         Primary non-Hodgkin
Abid D, Mallek S, et      immunocompromised     large B-cell lymphoma
al. Cardiac tamponade    male presented with     3rd-degree AV block
and paroxysmal third-   cardiac tamponade as
degree                   well as paroxysmal
atrioventricular           third-degree AV
block revealing a        block. He was found
primary cardiac non-     to have a large RV
Hodgkin large B-cell       mass. Following
lymphoma of the right    excision, histology
ventricle: a case          confirmed non-
report. J Med Case      Hodgkin large B-cell
Rep. 2011 Sep 5; 5:           lymphoma.
433 (English,

Houchaymi Z, Helou S,        78-year-old         Primary non-Hodgkin
Ballout J.              immunocompetent male    large B-cell lymphoma
Pericardial tamponade      presented with        3rd-degree AV block
and third-degree        pericardial tamponade
atrioventricular         and third-degree AV
block revealing a       block. Biopsy of the
primary cardiac         cardiac tumor showed
lymphoma. Rev Med       non-Hodgkin large B-
Interne. 2010 Nov; 31      Cell lymphoma.
(11): e4-6 (French,

Chiba Y, Oka K, Saito   49-year-old male with   Diffuse large B-cell
H, et al. Primary       cardiac tamponade and     lymphoma AV block
cardiac B-cell          AV block. Pericardial
lymphoma presented as       effusion and
heart tamponade and      transvenous biopsy
atrioventricular         confirmed diagnosis
block: a case report.    of diffuse large B-
Acta Cytol. 2010 Jan-     cell lymphoma. He
Feb; 54 (1): 79-81            underwent
(English, 20306995)       chemotherapy and
                         permanent pacemaker

Legault S, Couture C,   74-year-old male with     Burkitt-like PCL
Bourgault C, et al.     dyspnea found to have
Primary cardiac          a large pericardial
Burkitt-like lymphoma       effusion with
of the right atrium     tamponade physiology.
                        Found on pericardial
                          fluid studies and
                           biopsy to have
                          Burkitt-like PCL.

Ling LF, Chai P, Kee         55-year-old
AC, et al. Primary       immunocompetent man
cardiac lymphoma           presented with
presenting with         pyrexia initially and
cardiac tamponade. Am    was found later to
Heart Hosp J. 2009          have cardiac
Winter; 7 (2): E125-      tamponade. He was
7 (English, 19279985)    diagnosed with PCL.

Gosev I, Siric F,            67-year-old        Diffuse large B-cell
Gasparovic H, et al.       immunocompetent           lymphoma of
Surgical treatment of      presented with       centroblastic subtype
a primary cardiac         dyspnea. Found to      Surgical diagnosis,
lymphoma presenting        have tamponade       no pericardiocentesis
with tamponade              physiology on             performed
physiology (English,      echocardiography.
16846425)                  Surgical biopsy
                          confirmed diffuse
                        large B-cell lymphoma
                          of centroblastic

Wilhite DB, Quigley     83-year-old Filipino         Failure of
RL. Occult cardiac        male with dyspnea          traditional
lymphoma presenting           found on             pericardiostomy
with cardiac              echocardiogram to
tamponade. Tex Heart        have a large,
Inst. J. 2003; 30            homogenous
(1): 62-4 (English,     pericardial effusion
12638674)               with RA collapse. He
                          was treated with
                         pericardiostomy and
                         had the pericardial
                          drain removed the
                           next day as he
                        clinically improved.
                        He returned two weeks
                           later with same
                         symptoms and again
                            found to have
                          pericardial fluid
                          requiring urgent

Menotti A, Imperadore    Malignant lymphoma     Right atrial rupture
F, Pelosi G. et al.     presenting as cardiac   causing the tamponade
Heart rupture at the      tamponade due to
right atrial level as   right atrial rupture.
the first
manifestation of
malignant lymphoma.
Cardiologia. 1996
Jan; 41(1):65-7
(Italian, 8697472)

Roller MB, Manoharan      Elderly male with
A, Lvoff R. Primary       cardiac tamponade
cardiac lymphoma.       found on pericardial
Acta Haematol. 1991;      fluid analysis to
85 (1): 47-48                 have PCL.
(English, 2011932)

Nagamine K, Noda H.     Two cases of males in
Two cases cardiac       their 70s presenting
lymphoma presenting     with dyspnea who were
with pericardial            found to have
effusion and            pericardial effusion
tamponade. Jpn Circ.       with tamponade
J. 1990 Sep; 54 (9):        physiology on
1158-64 (English,          echocardiogram.

Pozniak AL, Thomas      Patient who presented
RD, Hobbs CB, et al.      with ventricular
Primary malignant        arrhythmias and was
lymphoma of the             found to have
heart. Antemorten       pericardial effusion
cytologic diagnosis.    with impairment of LV
Acta Cytol. 1986 Nov-       contraction.
Dec; 30 (6): 662-4.          Postmortem
(English, 3466501)       examination showed
                         malignant lymphoma
                             confined to

Patel J, Melly L,        Case series of six     Clinical presentation
Sheppard MN. Primary     patients presenting      of PCL (B-and T-
cardiac lymphoma: B-    with wide spectrum of          cells)
and T-cell cases at a   symptoms. Most cases
specialist UK centre     involved 2 or more
                           chambers of the
                        heart. Presentations
                         included conduction
                         effusion, valvular
                         dysfunction, heart
                        failure, stroke, and
                            sudden death.

Figure 5: Graphical representation of frequencies of subtypes of
non-Hodgkin's lymphoma.

Types of non -Hodgkin's

Primary Cardiac        10
Diffuse large B-cell   0.5
Follicular             20
Mantle cell            7
Small lymphocytic      5
Primary mediastinal    2.5
Lymphoplasmacytic      2
Marginal zone          10
Burkitt                0
T-cell lymphoma        2

Note: Table made from pie chart.
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Title Annotation:Case Report
Author:Quiroz, Elisa; Hafeez, Adam; Mando, Ramy; Yu, Zhou; Momin, Feroze
Publication:Case Reports in Oncological Medicine
Date:Jan 1, 2018
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