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Arch Cardiovasc Imaging. 2016 August; 4(3):e42537.
doi: 10.5812/acvi.42537.
Published online 2016 August 27.
Case Report
Primary Malignant Cardiac Tumor Presenting with Dyspnea: A Case
Report
Hedieh
Alimi,1 and Masumeh Alvandi2,*
1
MD, Assistant Professor of Echocardiography, Department of Cardiology, Cardiovascular Research Center, Quaem Hospital, Faculty of Medicine, Mashhad University of
Medical Sciences, Mashhad, IR Iran
2
MD, Fellow of Echocardiography, Department of Cardiology, Cardiovascular Research Center, Quaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences,
Mashhad, IR Iran
*
Corresponding author: Masumeh Alvandi, MD, Fellow of Echocardiography, Department of Cardiology, Cardiovascular Research Center, Quaem Hospital, Faculty of Medicine,
Mashhad University of Medical Sciences, Mashhad, IR Iran. Tel: +98-9155045734, E-mail: [email protected]
Received 2016 June 29; Revised 2016 July 02; Accepted 2016 July 31.
Abstract
Primary malignant cardiac tumors are rare tumors of the heart with a very poor prognosis. Complete excision is the treatment of
choice, but it is dependent on the stage and extension of the tumor. We describe an old man with the initial presenting symptoms
of progressive dyspnea. Our assessment revealed moderate pericardial effusion and a large infiltrative right ventricular mass. The
initial differential diagnosis included malignant sarcoma, lymphoma, or melanoma. The patient underwent palliative excision of
the tumor and chemotherapy. After biopsy, cardiac lymphoma was confirmed.
Keywords: Cardiac Lymphoma, Pericardial Effusion, Echocardiography
1. Introduction
Primary malignant cardiac tumors are rare tumors of
the heart and include 1% of primary cardiac tumors and
0.5% of extranodal lymphomas. They have a very poor
prognosis. Complete excision is deemed the treatment of
choice, although it is dependent on the stage and extension of the tumor (1, 2). We introduce a rare case of cardiac lymphoma with the initial presenting symptoms of
progressive dyspnea.
2. Case Presentation
A 64-year-old man was admitted to our center with
symptoms of dyspnea, having started 1 month previously
and progressed gradually from functional class II to III. The
patient’s vital signs were normal.
In physical examination, jugular vein pressure was elevated without any lower extremity edema and abdominal
distention. The chest roentgenogram showed small left
plural effusion (Figure 1).
ECG showed sinus rhythm with a low-voltage QRS amplitude and a right bundle branch block pattern, q and S-T
elevation, and inverted T waves in V1 - V4 and the inferior
leads.
Echocardiography revealed severe right ventricular
(RV) enlargement and systolic dysfunction with a large (8
cm × 5 cm) nonhomogeneous lobulated mass in the lateral and inflow parts of the RV with invasion to the RV myocardium, protruding into the RV outflow tract and even
Figure 1. Chest roentgenogram shows small left plural effusion (black arrow).
the pulmonary valve during systole. There was large-sized
pericardial effusion without evidence for the physiology of
tamponade (3) (Figure 2).
Left ventricular size and function were normal, and no
mass was detected on the left side. Moderate central tricuspid regurgitation with right atrial enlargement was also
Copyright © 2016, Iranian Society of Echocardiography. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0
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Alimi H and Alvandi M
3. Discussion
Primary cardiac lymphomas constitute very rare malignant cardiac tumors with the involvement of only the
heart and the pericardium. The Non-Hodgkin type involves
the heart and the pericardium and, less commonly, extracardiac sites. The right atrium and the RV are frequently
involved. Primary cardiac lymphomas include about 1%
of primary cardiac tumors and 0.5% of extranodal lymphomas. They range in age from 18 to 77 years with equal
sex distribution. Clinically, cardiac tumors cause symptoms in patients by 3 mechanisms: 1) intracardiac obstruction, 2) systemic embolization of tumor fragments, and
3) constitutional symptoms (1, 2). The symptoms are related to the site of involvement in the heart (4). Lymphoma
should be suspected when patients present with a cardiac
mass or unexplained refractory pericardial effusion and
when more than 1 chamber is involved (5). In our patient,
the RV was involved with protrusion into the main pulmonary artery and invasion to the pericardium (with no
evidence of extra-cardiac involvement), causing dyspnea.
The treatment of choice for these rare tumors is a combination of chemotherapy and radiation, but this modality
is dependent upon the stage and the extent of infiltration
at presentation (3). The survival is generally less than a
month without treatment but has been prolonged for up
to 5 years with palliative treatment in selected cases (1).
Footnote
Conflict of Interest: There is no conflict of interest.
Figure 2. 2D echocardiography shows a large mass in the RV free wall in images A (4chamber view) and B (short-axis view), marked by the white arrow. Large-sized pericardial effusion (2.6 cm) is shown in image A by the yellow arrow. RA, Right atrium;
LA, Left atrium; LV, Left ventricle; IVS, Interventricular septum.
found. The inferior vena cava was dilated with reduced respiratory collapse without any obvious mass in it. Based
on these data, our initial differential diagnosis included
primary or secondary cardiac malignant tumors such as
metastatic sarcoma, melanoma, or lymphoma.
Abdominal computed tomography (CT) scan and thoracic CT angiography showed RV mass and left pleural effusion without evidence for pulmonary emboli or the involvement of the other organs.
Finally, cardiothoracic consult was done and the patient was transferred to the operating room for the palliative excision of the tumor. Tumor biopsy revealed atypical, intermediate to large-sized lymphoid cell infiltration
(Figure 3). The final diagnosis was primary diffuse cardiac
large B-cell lymphoma, and the patient was subsequently
referred for chemotherapy.
2
Arch Cardiovasc Imaging. 2016; 4(3):e42537.
Alimi H and Alvandi M
Figure 3. Tumor is composed of atypical, intermediate to large-sized lymphoid cell infiltration, leading to the final diagnosis of diffuse large B-cell lymphoma.
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