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E-JCRT CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1034

Primary cardiac lymphoblastic B-cell lymphoma: Should we treat more intensively?


1 Post Graduate Program in Medical Science, Department of Clinical Medicine, Federal University of Ceara, Fortaleza, Brazil
2 Health Sciences Center, University of Fortaleza, Fortaleza, Ceara, Brazil

Date of Web Publication15-Feb-2016

Correspondence Address:
Ronald Feitosa Pinheiro
R. Pereira Valente, 738, Meireles, 60160250, Fortaleza, Ceará
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.154063

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 > Abstract 

Primary cardiac lymphoma (PCL) is a rare neoplasm, the majority of cases of which are non-Hodgkin's, diffuse large B-cell (DLBCL). We report the first case of an adult with PCL B-cell lymphoblastic lymphoma whose disease evolution was grim. A 52-year-old male reported dyspnea and facial swelling lasting for 4 months and upon a physical examination he presented bradycardia, jugular venous engorgement, and hypophonesis of cardiac sounds. An electrocardiography (Echo) revealed a right atrial mass and nodules at the pericardium. The patient was treated with R-Hyper-CVAD (rituximab plus cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and presented very short remission. At this time, we used R-ICE (rituximab plus ifosfamide, carboplatin, and etoposide) chemotherapy and the patient underwent complete remission after two courses and received autologous bone marrow transplantation (auto-BMT). After 75 days of follow-up, the patient reported dyspnea and a new Echo showed a recurrence of the disease. The patient died due to cardiac failure. PCL is a rare disease with an unfavorable prognosis and a prompt diagnosis and treatment are fundamental to survival. We believe that more intensive therapies, such as auto-BMT, should be considered as a first treatment option.

Keywords: B-cell, lymphoblastic lymphoma, primary cardiac lymphoma, treatment


How to cite this article:
Pires Ferreira Filho LI, Ribeiro HL, Pinheiro ED, Pinheiro RF. Primary cardiac lymphoblastic B-cell lymphoma: Should we treat more intensively?. J Can Res Ther 2015;11:1034

How to cite this URL:
Pires Ferreira Filho LI, Ribeiro HL, Pinheiro ED, Pinheiro RF. Primary cardiac lymphoblastic B-cell lymphoma: Should we treat more intensively?. J Can Res Ther [serial online] 2015 [cited 2019 Sep 20];11:1034. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1034/154063


 > Introduction Top


Primary cardiac lymphoma (PCL) is a rare neoplasm, the majority of cases of which are non-Hodgkin's, diffuse large B-cell (DLBCL) involving the pericardium, right atrium, and right ventricle. [1],[2],[3],[4] PCL is detected in 1-3% of cardiac tumors, representing less than 0.5% of extranodal lymphomas. [3],[4] These rarely reported cases demonstrate involvement of the right heart, in contrast to myxoma, which typically involves the left atrium. [1],[3],[4]

A prompt diagnosis of PCL is important to achieve a complete response and is fundamental to favorable evolution of the disease. [2],[3],[4],[5],[6],[7] A diagnosis is often difficult since PCL manifests symptoms consistent with other cardiopulmonary disorders. Many patients report dyspnea for months before diagnosis and this delay decreases the chance of curing the disease. [2],[3],[4],[5],[6],[7]

The aim of this report is to present, to the best of our knowledge, the first case of an adult with PCL B-cell lymphoblastic lymphoma whose disease evolution was grim. We reviewed the reported cases of the last 10 years; evaluating the clinical presentation, evolution, and response to the treatment.


 > Case report Top


A 52-year-old male reported dyspnea and facial swelling lasting for 4 months. Upon a physical examination, he presented bradycardia, jugular venous engorgement, and hypophonesis of cardiac sounds. An electrocardiography (Echo) revealed sinus bradycardia. The Echo also revealed a right atrial mass and nodules at the pericardium. The patient underwent a biopsy of his nodules in the pericardium/myocardium [Figure 1]a and b] and a diagnosis of B lymphoblastic lymphoma (CD20 (+), CD79a (+), TdT (+), CD3 (−), and CD5 (−)) was established (World Health Organization). Positron emission tomography-CT (PET-CT) showed a markedly increased fluorodeoxyglucose (FDG) uptake across the pericardium/myocardium without uptake in other lymph nodes [Figure 1]a and b].
Figure 1: (a) Lymphoblastic lymphoma showing an extensive number of nodules in the pericardium/myocardium. (b and c) PET-CT showed a markedly increased FDG uptake across the pericardium/myocardium without uptake in other lymph nodes. The arrow indicates the nodules PET-CT = Positron emission tomography-computed tomography, FDG = fluorodeoxyglucose

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The patient was treated with R-Hyper-CVAD course A (rituximab plus cyclophosphamide, vincristine, doxorubicin and dexamethasone) and course B (high dose of methotrexate and cytarabine) plus rituximab. After the second course of chemotherapy, the patient exhibited a complete remission according to an Echo, but the disease progressed shortly after the third course. An Echo demonstrated once again revealed nodules at the pericardium/myocardium. At this time, we used R-ICE chemotherapy, a combination of rituximab plus ifosfamide, carboplatin, and etoposide. The patient underwent complete remission after two courses and received autologous bone marrow transplantation (auto-BMT). After 75 days of follow-up, the patient reported dyspnea and a new Echo showed a recurrence of the disease. The patient died due to cardiac failure.


 > Discussion Top


The diagnosis of PCL is extremely rare. Recently, Cresti et al., [8] evaluated the incidence of primary cardiac tumors in a 14-year population study. The researchers detected 1.38 cases per year in 100,000 individuals. The most commonly detected tumors were myxoma, fibroelastoma, lipoma, rhabdomyoma, hemangioma, sarcoma, and lymphoma, representing 48, 15, 15, 8, 5, 5, and 2% of cases, respectively. Davis et al., [9] also studied the Surveillance, Epidemiology, and End Results (SEER) experience with primary pediatric cardiac malignancies. They detected an age-adjusted incidence of 0.00686 per 100,000 individuals in the United States. The most common subtype was soft sarcoma, followed by non-Hodgkin's lymphoma.

To the best of our knowledge, the case studied here is the first report of an adult with primary cardiac B-cell lymphoblastic lymphoma. This disorder is highly aggressive with a predominance of T-cell lineage and commonly presents a bulky anterior mediastinal mass, pleural effusion, or organomegaly. [10] After reviewing the literature, we detected only one case of primary B-Cell lymphoblastic lymphoma. [11] The case was a 10-year-old child who presented with fatigue , syncope, and vomiting for 2 weeks before diagnosis. [11] The most common subtype of PCL is DLBCL, [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] which is detected in more than 50% of cardiac lymphomas, followed by low-grade B cell lymphoma [Table 1].
Table 1: Reported cases of patients with primary cardiac lymphoma with clinical presentation, time to diagnosis, response to treatment and survival


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The majority of patients with PCL present symptoms related to cardiac frequency or difficulty breathing. [2],[3],[4],[5],[6] Dyspnea is the most common symptom that may be related to pleural effusion. [2],[3],[7],[19],[20] Patients occasionally report a history of several months of progressive fatigue, night sweats, and weight loss before their diagnosis. We detected a case with a 9-month delay prior to diagnosis. [21] Our patient reported progressive dyspnea for 4 months before diagnosis. Arrhythmias and syncope are commonly detected. [11],[19],[20] The most common arrhythmias are atrial fibrillation, atrial flutter, and bradycardia [Table 1]. [19],[20] Our patient presented with sinus bradycardia which was related to the involvement of the right atrium. The sinoatrial node (located in the right atrium) is the natural pacemaker that initiates impulses for the heartbeat; if it is blocked by lymph nodes, its normal function can be disrupted. As cases of arrhythmia are evaluated, an Echo must always be performed. If the Echo demonstrates any mass in the right atrial, the chance of lymphoma increases because atrial myxoma typically involves the left atrium. [19],[20] Of utmost importance, we also detected two patients who were initially diagnosed as having hypertrophic cardiomyopathy, but after biopsy were diagnosed as non-Hodgkin's lymphoma. [16],[21]

The majority of patients have been treated with CHOP/R-CHOP (rituximab, cyclophosphamide, adriamycin, vincristine, and prednisone) with variable response [2],[5],[6],[7],[19] and many cases present very short (less than one month) survival durations [2],[19] with conventional chemotherapy [Table 1]. [6],[19],[20] Long-term survivalis observed when patients were treated with more intensive therapy as Berlin-Frankfurt-Munster (BFM), anaplastic large cell lymphoma (ALCL 99), and auto-BMT: 24, 29, and 24 months, respectively. Unfortunately, our patient exhibited a quick recurrence of the disease after auto-BMT. We suggest that intensive therapies, including BFM, ALCL 99, and auto-BMT, should be considered as a first line for treatment of patients with more extensive cardiac involvement, such as biatrial and biventricular involvement.

Surgery should not be considered the only treatment. Non-Hodgkin's lymphoma is a systemic disorder and chemotherapy should always be used as treatment. We detected patients who were treated with surgery out of necessity of maintaining cardiac function, but the best results were reported when chemotherapy was added as an adjunct to surgery . [17],[18]

Sometimes, surgery is not an option of treatment because the myocardium is too rigid due to tumor invasion of the pericardium and myocardium. Modern imaging modalities, including Magnetic Resonance Imaging ( MRI), may help to determine the extent of tumor involvement in the heart. Surgery for mass reduction is not usually recommended because it does not seem to improve the prognosis. [7] The most common approach is pericardiocentesis, which is performed urgently to remove pericardial effusion. [7],[10],[15] Literature review did not reveal any PCL treated with radiotherapy . This modality of treatment is likely dangerous to the heart due to increased chance of arrhythmias and fibrosis of pericardium.

PCL is a disease with an unfavorable prognosis. [2],[5],[19],[20] Many patients present symptoms related to cardiac frequency, especially arrhythmias or difficulty in breathing and a prompt diagnosis and treatment are fundamental to survival. Many cases present very short survival durations with conventional chemotherapy (CHOP/R-CHOP) and we believe that more intensive therapies, such as auto-BMT, should be considered as a first treatment option.

 
 > References Top

1.
Abdullah HN, Nowalid WK. Infiltrative cardiac lymphoma with tricuspid valve involvement in a young man. World J Cardiol 2014;6:77-80.  Back to cited text no. 1
    
2.
Soens L, Schoors D, Van Camp G. Acute heart failure due to fulminant myocardial infiltration by a diffuse large B-cell lymphoma. Acta Cardiol 2012;67:101-4.  Back to cited text no. 2
    
3.
Cho JS, Her SH, Park MW, Kim HD, Baek JH, Youn HJ, et al. A butterfly-shaped primary cardiac lymphoma that showed bi-atrial involvement. Korean Circ J 2012;42:46-9.  Back to cited text no. 3
    
4.
Smith M, Golwala H, Magharyous H, Trotter T, Sawh R, Lozano P. Right atrial B-cell lymphoma in a patient with ocular melanoma. J Card Surg 2011;26:625-8.  Back to cited text no. 4
    
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Lin JN. Cardiaclymphoma with first manifestation of recurrent syncope-a case report and literature review. Int Med Case Rep J 2010;10:1-6.  Back to cited text no. 5
    
6.
Mangasarova IA, Magomedova AU, Kravchenko SK, Zvonkov EE, Kremenetskaia AM, Vorob'ev VI, et al. Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: Diagnosis and treatment. Ter Arkh 2010;82:61-5.  Back to cited text no. 6
    
7.
Chin JY, Chung MH, Kim JJ, Lee JH, Kim JH, Maeng IH, et al. Extensive primary cardiac lymphoma diagnosed by percutaneous endomyocardial biopsy. J Cardiovasc Ultrasound 2009;17:141-4.  Back to cited text no. 7
    
8.
Cresti A, Chiavarelli M, Glauber M, Tanganelli P, Scalese M, Cesareo F, et al. Incidence rate of primary cardiac tumors: A 14-year population study. J Cardiovasc Med 2014.  Back to cited text no. 8
    
9.
Davis JS, Allan BJ, Perez EA, Neville HL, Sola JE. Primary pediatric cardiac malignancies: The SEER experience. Pediatr Surg Int 2013;29:425-9.  Back to cited text no. 9
    
10.
Thomas DA, O'Brien S, Cortes J, Giles FJ, Faderl S, Verstovsek S, et al. Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma. Blood 2004;104:1624-30.  Back to cited text no. 10
    
11.
Bassi D, Lentzner BJ, Mosca RS, Alobeid B. Primary cardiac precursor B lymphoblastic lymphoma in a child: A case report and review of the literature. Cardiovasc Pathol 2004;13:116-9.  Back to cited text no. 11
    
12.
Anghel G, Zoli V, Petti N, Remotti D, Feccia M, Pino P, et al. Primary cardiac lymphoma: Report of two cases occurring in immunocompetent subjects. Leukemia Lymphoma 2004;45:781-8.  Back to cited text no. 12
    
13.
Rolla G, Bertero MT, Pastena G, Tartaglia N, Corradi F, Casabona R, et al. Primary lymphoma of the heart. A case report and review of the literature. Leuk Res 2002;26:117-20.  Back to cited text no. 13
    
14.
Cho SW, Kim BK, Hwang JT, Kim JH, Kim BO, Goh CW, et al. A case of primary cardiaclymphoma mimicking acute coronary and aortic syndrome. Korean Circ J 2012;42:776-80.  Back to cited text no. 14
    
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Lauten M, Vieth S, Hart C, Wössmann W, Tröger B, Härtel C, et al. Cardiac anaplastic large cell lymphoma in an 8-year old boy. Leuk Res Rep 2014;3:36-7.  Back to cited text no. 15
    
16.
Lee GY, Kim WS, Ko YH, Choi JO, Jeon ES. Primary cardiaclymphoma mimicking infiltrative cardiomyopathy. Eur J Heart Fail 2013;15:589-91.  Back to cited text no. 16
    
17.
Habertheuer A, Ehrlich M, Wiedemann D, Mora B, Rath C, Kocher A. A rare case of primary cardiac B cell lymphoma. J Cardiothorac Surg 2014;9:14.  Back to cited text no. 17
    
18.
Donegani E, Ambassa JC, Mvondo C, Giamberti A, Ramponi A, Palicelli A, et al. Primary cardiac Burkitt lymphoma in an African child. G Ital Cardiol 2013;14:481-4.  Back to cited text no. 18
    
19.
Patel J, Melly L, Sheppard MN. Primary cardiac lymphoma: B- and T-cell cases at a specialist UK centre. Ann Oncol 2010;21:1041-5.  Back to cited text no. 19
    
20.
Nascimento AF, Winters GL, Pinkus GS. Primary cardiac lymphoma: Clinical, histologic, immunophenotypic, and genotypic features of 5 cases of a rare disorder. Am J Surg Pathol 2007;31:1344-50  Back to cited text no. 20
    
21.
Kim DH, Kim YH, Song WH, Ahn JC. Primary cardiaclymphoma presenting as an atypical type of hypertrophic cardiomyopathy. Echocardiography 2014;31:E115-9.  Back to cited text no. 21
    


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