|Year : 2015 | Volume
| Issue : 4 | Page : 1034
Primary cardiac lymphoblastic B-cell lymphoma: Should we treat more intensively?
Luiz Ivando Pires Ferreira Filho1, Howard Lopes Ribeiro1, Edílson DiÓgenes Pinheiro2, Ronald Feitosa Pinheiro1
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 Publication||15-Feb-2016|
Ronald Feitosa Pinheiro
R. Pereira Valente, 738, Meireles, 60160250, Fortaleza, Ceará
Source of Support: None, Conflict of Interest: None
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|| |
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. ,,, PCL is detected in 1-3% of cardiac tumors, representing less than 0.5% of extranodal lymphomas. , These rarely reported cases demonstrate involvement of the right heart, in contrast to myxoma, which typically involves the left atrium. ,,
A prompt diagnosis of PCL is important to achieve a complete response and is fundamental to favorable evolution of the disease. ,,,,, 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. ,,,,,
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|| |
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|| |
The diagnosis of PCL is extremely rare. Recently, Cresti et al.,  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.,  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.  After reviewing the literature, we detected only one case of primary B-Cell lymphoblastic lymphoma.  The case was a 10-year-old child who presented with fatigue , syncope, and vomiting for 2 weeks before diagnosis.  The most common subtype of PCL is DLBCL, ,,,,,,,,,,, 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. ,,,, Dyspnea is the most common symptom that may be related to pleural effusion. ,,,, 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.  Our patient reported progressive dyspnea for 4 months before diagnosis. Arrhythmias and syncope are commonly detected. ,, The most common arrhythmias are atrial fibrillation, atrial flutter, and bradycardia [Table 1]. , 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. , 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. ,
The majority of patients have been treated with CHOP/R-CHOP (rituximab, cyclophosphamide, adriamycin, vincristine, and prednisone) with variable response ,,,, and many cases present very short (less than one month) survival durations , with conventional chemotherapy [Table 1]. ,, 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 . ,
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.  The most common approach is pericardiocentesis, which is performed urgently to remove pericardial effusion. ,, 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. ,,, 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.
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