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| INTERESTING CASE REPORT |
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| Year : 2023 | Volume
: 7
| Issue : 1 | Page : 54-58 |
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Primary Cardiac Lymphoma: Case Series
Sidhant Sachdeva1, Rohit Tandon2, Rajiv Gupta3, Davinder Paul4
1 Fellow in Non Invasive Cardiology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
2 Consultant Physician, Dayanand Medical College and Hospital, Unit Hero DMC Heart Institute, Ludhiana, Punjab, India
3 Department of CTVS, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
4 Department of Oncology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
| Date of Submission | 24-Sep-2022 |
| Date of Decision | 02-Nov-2022 |
| Date of Acceptance | 10-Nov-2022 |
| Date of Web Publication | 30-Mar-2023 |
Correspondence Address:
Dr. Rohit Tandon
Hero DMC Heart Institute, Ludhiana - 141 001, Punjab
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jiae.jiae_49_22
Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is frequently found in immunocompromised patients and is fatal unless timely diagnosed and treated. Untreated, it has a mean survival time of 6 months. The differential diagnosis includes other malignant cardiac neoplasms. Among the imaging modalities, echocardiography is emerging as an indispensable investigation for the early diagnosis and follow-up of these patients, although histopathology gives the final diagnosis. Here, we present three cases of PCL in immunocompetent patients, out of whom two were adults and one was a child. Two patients presented with pericardial tamponade and a third with complete heart block. On echocardiography, lymphoma was located in the right heart chambers in all three cases with the presence of significant pericardial effusion. Histopathological examination showed diffuse large B-cell lymphoma in adults and T-cell lymphoblastic lymphoma in the child. All three patients received chemotherapy. Only two patients survived. The child remained asymptomatic, while the adult male had a relapse after 6 months of completion of the first chemotherapy regimen. The diagnosis of PCL is difficult due to nonspecific clinical manifestations. At present, the main course of treatment is chemotherapy, although surgery may be required in some cases. Among the available imaging modalities, echocardiography is a cost-effective and readily available investigation for the early detection of the tumor.
Keywords: Echocardiography, multimodality imaging, primary cardiac lymphoma
How to cite this article:
Sachdeva S, Tandon R, Gupta R, Paul D. Primary Cardiac Lymphoma: Case Series. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:54-8 |
How to cite this URL:
Sachdeva S, Tandon R, Gupta R, Paul D. Primary Cardiac Lymphoma: Case Series. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 May 29];7:54-8. Available from: https://jiaecho.org/text.asp?2023/7/1/54/373011 |
| Introduction | |  |
Lymphomas constitute a heterogeneous group of malignant disorders with protean clinical manifestations, pathologic features, and imaging characteristics. Primary cardiac lymphoma (PCL) is a rare malignant disease comprising only 1.3% of all cardiac tumors and 0.5% of all extranodal lymphomas.[1],[2] They are more commonly seen in immunocompromised patients. The most common histological type is large B-cell lymphoma. The typical location is the right atrium.
| Case Reports | | |
Case 1
A 72-year-old woman was referred to the cardiology unit for the evaluation of syncope resulting in a hip fracture. Twelve-lead electrocardiogram revealed a complete heart block and a heart rate of 40 beats/min. Transthoracic echocardiography indicated the presence of a large right atrial (RA) polypoid mass attached to the interatrial septum and right atrioventricular groove, with nonopacification on myocardial contrast echocardiography [Figure 1] [Video 1]. Temporary pacing was urgently done through the transfemoral route, followed by a transvenous biopsy of the right heart mass under simultaneous fluoroscopic and echocardiographic guidance. Microscopically, the tumor consisted of a dense proliferation of large- and intermediate-sized cells invading myocardial muscle bundles. Cell nuclei had irregular chromatin with several nucleoli and basophilic cytoplasm indicative of diffuse large B-cell lymphoma [Figure 2]. She tested negative for human immunodeficiency virus (HIV) infection. Bone marrow biopsy showed no systemic lymphogenous involvement. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was administered, but the patient succumbed to her illness after the third chemotherapy cycle because of pneumonia. | Figure 1: Case 1: 12 lead electrocardiogram showing complete heart block
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 | Figure 2: Case 1: Transthoracic echocardiography images showing (a) subcostal view with red arrow pointing towards the mass in the atrioventricular (AV) groove, black arrow pointing towards the thickened right ventricle free wall and yellow arrow pointing towards the thickened interatrial septum. (b) Contrast image of the same patient in the subcostal view with arrow pointing towards the mass in the AV groove
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[Additional file 1]
Video 1: Case 1- Subcostal view on transthoracic contrast echocardiography showing a mass in the atrioventricular groove.
Case 2
A 65-year-old male was admitted with fever, myalgia, and swelling at the back of the neck for 1 month and increasing breathlessness for 2 weeks. Chest X-ray revealed cardiomegaly. Transthoracic echocardiography showed moderate-to-large pericardial effusion with sessile hypoechoic masses along the RA, right atrioventricular (AV) groove, and right ventricular (RV) free wall [Figure 2] [Video 2] and [Video 3]. These masses lacked opacification on myocardial contrast imaging. After pericardiocentesis, fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) and trucut biopsy revealed FDG-avid nodular, ill-defined, enhancing soft-tissue masses, measuring 34 cm along the free wall of the RA, RV, right AV groove, and interatrial septum with no other FDG-avid mass elsewhere in the body [Figure 3]. Gross examination of the masses revealed a large round tumor suggestive of malignant etiology, possibly non-Hodgkin lymphoma (NHL). Microscopically, malignant round cells with irregular nuclear contours and scant neoplasm were seen. Immunohistochemistry was strongly positive for CD45 and CD20 confirming activated B-cell type NHL [Figure 4]. Abdominal ultrasonography, bone marrow biopsy, and HIV antibody were done to rule out other diseases. Eight cycles of chemotherapy were administered. This included cyclophosphamide, vincristine, procarbazine, and prednisone. The patient remained in complete clinical remission for 6 months on follow-up. After 6 months, he started complaining of progressive neck swelling with constitutional symptoms. Biopsy of the neck swelling was suggestive of recurrence of malignancy. He was retreated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone for three cycles, followed by a course of 40 Gy mediastinal irradiation. He achieved clinical remission which was confirmed by PET/CT till 12-month follow-up. | Figure 3: Case 1: 400x hematoxylin and eosin stained sections showing sheets of atypical lymphoid cells
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 | Figure 4: Case 2: (a) Transthoracic echocardiography showing moderate to large pericardial effusion with sessile hypo echoic masses along right atrium, atrioventricular groove, and right ventricular free wall. (b) after completion of chemotherapy showing complete tumor reduction
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[Additional file 2]
Video 2: Case 2- Apical four-chamber showing moderate-to-large pericardial effusion with sessile hypoechoic masses along the right atrium, atrioventricular groove, and right ventricular free wall.
[Additional file 3]
Video 3: Case 2- Subcostal view of the same patient showing moderate-to-large pericardial effusion with sessile hypoechoic masses along right atrium, atrioventricular groove, and right ventricular free wall.
Case 3
A 4-year-old boy was referred to the cardiology department for urgent relief of pericardial tamponade. He gave a history of fever for 2 months and progressive breathlessness for 4 days. Transthoracic echocardiography revealed multiple echogenic masses along RA and RV-free walls along with pericardial involvement [Figure 5] [Video 4]. After pericardiocentesis, the masses were biopsied in three pericardial approaches. The hematoxylin- and eosin-stained sections showed diffuse infiltration by atypical cells having a high nuclear-to-cytoplasmic ratio and scanty cytoplasm suggestive of T-cell lymphoblastic lymphoma [Figure 6] which was positive for CD7 on immunohistochemistry [Figure 7]. The patient was started on chemotherapy and treated with six cycles of vincristine, cyclophosphamide, doxorubicin, prednisone, ranimustine, vindesine, etoposide, and carboplatin. After three chemotherapy cycles, there was a significant reduction in the size of the tumor on echocardiography and at 6-month follow-up, he remained in complete clinical remission. | Figure 5: Case 2: Positron emission tomography/computed tomography images of the patient showing increased fluorodeoxyglucose uptake along right ventricular and left ventricular free walls
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 | Figure 6: Case 2: Histological section from the cardiac mass reveals diffuse infiltration of lymphoid cells
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 | Figure 7: Case 3: Subcostal four-chamber view showing non opacifying mass along right atrial wall on myocardial contrast echocardiography
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[Additional file 4]
Video 4: Case 3- Subcostal four-chamber view showing a mass along the right atrial wall on transthoracic echocardiography.
| Discussion | | |
Primary malignant cardiac tumors are rare. By reviewing the data of 22 autopsy series, Reynen[3] found a prevalence of 0.02%. PCL is even more unusual. Its prevalence varies from 1% to 2% of primary cardiac tumors.[4] PCL is an extranodal malignant lymphoma of any cell type involving only the heart and pericardium without dissemination.
The usual age at the presentation is 62 ± 4 years with a male predominance (2:1). Bone marrow and superficial lymph nodes are usually not involved, although mediastinal lymphadenopathy may be detected. PCL in children is exceedingly rare. The incidence and prevalence of PCL are not described in the literature as most of the studies included middle-aged individuals.
The disease remains asymptomatic until it produces a mass effect affecting cardiac output or local invasion causes AV block. Chest X-ray findings are nonspecific and may include pleural effusion and/or cardiomegaly. Transthoracic echocardiography is an excellent modality for the detection and monitoring of therapeutic results. The tumor is usually located along the right AV groove and RV free wall with/without involving the interatrial septum or pericardium. In most of the cases, tumor appears as sessile hyperechoic masses with the regular borders and no valvular involvement[5]. Involvement of the interatrial septum is usually diffuse. Myocardial contrast is helpful for cavity opacification. The tumor is identified as a nonopacifying mass, both at the baseline and in subsequent studies to identify the tumor location, size, and extent for assessing tumor reduction, and finally to document remission. Transesophageal echocardiography is helpful during surgical resection and biopsy. The tumor is unifocal in about 66% of cases and multifocal in 24% with an average size of 46 cm.
In our three patients, the tumor had a predilection for right heart chambers, a finding similar to the reviewed cases (69%). However, the reason for this distribution is not known.[6] This right-side predominance is in contrast to the left-side predominance in cardiac myxoma.[7] All three of our patients had stage II disease according to the revised staging system for primary nodal lymphomas (Lugano classification) [Table 1].[8] | Table 1: Revised staging system for primary nodal lymphomas (Lugano classification)[8]
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Drainage of pericardial effusion mainly has therapeutic benefits but little diagnostic significance. A diagnostic cytologic sample is obtained only in 14% of the cases. In the two adult patients, transvenous biopsies under simultaneous fluoroscopic and echocardiographic guidance were used for the diagnosis. Because of the thick appearing pericardial fluid on echocardiography, surgical pericardiocentesis was done and a biopsy was obtained from the mass along the RV apex.
Almost 85% of the reported cases had diffuse large B-cell lymphoma; rest of them had small cell lymphomas and T-cell lymphomas. Late diagnosis appears to be a major factor in the poor outcome in these patients.[4] Prompt anthracycline-based chemotherapy results in complete remission in 60% of the cases. Radiotherapy combined with chemotherapy further improves the survival. PCL should be treated similar to bulky aggressive lymphomas arising in other primary sites.[9],[10]
| Conclusion | | |
Multimodality imaging is the diagnostic approach of choice for patients with cardiac lymphomas. Echocardiography remains an important first-line investigation. This is because most of the patients with cardiac lymphomas are brought to attention as a result of pericardial tamponade, and the echocardiographic examination raises the suspicion of the underlying malignancy. Immediate pericardiocentesis and simultaneous tissue sampling under echocardiographic guidance assist in the early symptom resolution and further work-up. It is also a cost-effective investigation for the serial follow-up of the tumor. Myocardial contrast imaging is a useful adjunct as it delineates the space-occupying lesion, thereby defining the extent of involvement.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
[Table 1]
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