Primary Cardiac Lymphoma Research Articles

Syncope as a clinical presentation of cardiac lymphoma: a case report

Primary cardiac lymphoma, an extremely rare type of B-cell non-Hodgkin lymphoma, has an incidence of approximately 0.02-2% among malignant cardiac tumors. It is frequently associated with manifestations such as cardiac rhythm disturbances, refractory pericardial effusion and cardiac masses. Definitive diagnosis is achieved by biopsy, being essential to rule out hematogenous dissemination with PET-CT. The literature indicates a predominance of right chamber involvement and survival that varies significantly according to left ventricular involvement and the presence of arrhythmias. Therapeutic options are not well defined, but chemotherapy such as R-CHOP has shown efficacy, although the prognosis is generally poor due to complications such as disease progression, arrhythmias and sepsis. This case underscores the importance of considering cardiac lymphoma in patients with unexplained cardiac symptoms, highlighting the need for a multidisciplinary and specific diagnostic and therapeutic approach.

Treatment of malignant primary cardiac tumors requires attention to cardiovascular complications: a single-center, retrospective study.

Malignant primary cardiac tumors require multimodal approaches including surgery, chemotherapy and radiotherapy, but these treatments can be associated with cardiovascular complications. However, few reports have described the cardiovascular complications related to primary cardiac tumor treatment because of their rarity. Clinical records of patients with primary cardiac tumors treated at Kyushu University Hospital from January 2010 to August 2021 were retrospectively examined. Of the 47 primary cardiac tumor patients, 13 (28%) were diagnosed with malignancy, including 5 angiosarcomas, 3 intimal sarcomas, 3 diffuse large B-cell lymphomas, 1 Ewing's sarcoma and 1 fibrosarcoma. Cardiovascular events were observed in 10 patients (77%), including cardiac dysfunction in 6 patients, arrhythmias in 5 patients, right heart failure in 2 patients, and excessively prolonged prothrombin time due to the combination of warfarin and chemotherapy in 1 patient. Two patients who showed notable cardiac complications are described. Case A involved a 69-year-old woman who underwent surgery for a left atrial intimal sarcoma, followed by postoperative chemotherapy with doxorubicin plus ifosfamide and radiotherapy. After three cycles of chemotherapy and sequential radiotherapy, her left ventricular ejection fraction decreased to 34%, and ongoing heart failure therapy was required. Case B involved a 66-year-old man who received chemotherapy for primary cardiac lymphoma, resulting in tumor shrinkage. However, due to tumor involvement of the intraventricular septum, atrioventricular block developed, requiring cardiac pacemaker implantation. High incidences of cardiac failure and arrhythmias were observed during multimodal treatments for malignant primary cardiac tumors. Proper management of complications may lead to a favorable prognosis in patients with malignant primary cardiac tumors.

Primary cardiac lymphoma causing coronary artery compression and massive tricuspid regurgitation.

Primary cardiac lymphoma causing coronary artery compression and massive tricuspid regurgitation.

Cardiac Manifestations of Lymphoma: A Review of Primary and Secondary Cardiac Lymphoma.

Primary cardiac lymphoma (PCL) is a very unique and uncommon disease presentation, with reports in the literature limited to case reports. Most often it is B-cell in origin, predominantly diffuse large B-cell lymphoma. Symptomatic presentation of PCL depends on the location of anatomic involvement, but most often involves the right heart, with presentation consistent with heart failure, pericardial effusions, and atrioventricular nodal blockade. Endomyocardial biopsy is necessary for diagnosis, but cardiac magnetic resonance imaging has been the most useful for staging of the disease. The disease has a poor prognosis but treatment with chemotherapy has been the most successful approach. Particularly, the chemotherapy regimen of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone has been reported to be successful for diffuse large B-cell lymphoma, so it is often utilized first. In newer reports of patients with PCL, there may be a role of autologous stem cell transplant along with consolidative chemotherapy in younger patients diagnosed with PCL. Secondary cardiac lymphoma (SCL) is a more common occurrence that is often asymptomatic and recognized after the patient has passed from either the primary lymphoma or some other reason. Unlike PCL, SCL is more expansive and not often confined to the right heart. However, in patients with SCL who do have cardiac symptoms, the diagnostic approach and treatment are similar to that of PCL.

CASE-SB-03 - A Usual Presentation Resulting in an Unusual Diagnosis: A Case Report of a Primary Cardiac Lymphoma

CASE-SB-03 - A Usual Presentation Resulting in an Unusual Diagnosis: A Case Report of a Primary Cardiac Lymphoma

An Assessment of Primary Cardiac Lymphoma: A Rare Case in Indonesia

Introduction: : Primary cardiac lymphoma (PCL) is an uncommon malignancy with a high mortality rate. The disease is commonly found as non-Hodgkin’s lymphoma (NHL), mainly located in the heart or pericardium. PCL is difficult to diagnose due to its unspecific clinical manifestations. Echocardiography, computed tomography (CT) scanning, and magnetic resonance imaging (MRI) can help in diagnosing PCL, but histopathological examination is the gold standard for a definitive diagnosis. Therefore, this study aimed to compare the case of malignant cardiac lymphoma and MRI with earlier related literature. Case Presentation: This study reported a case of PCL in 52 years old male who came to Cipto Mangunkusumo Hospital with intra cardiac tumour and underwent surgery with median sternotomy. CT scan of the chest showed a mass in the left atrial and no significant abnormalities in other organs. Histopathological examination showed the morphologic feature of diffuse monotonous proliferation of atypical large B lymphocytes. The lymphoma diagnosis was supported by immunohistochemistry including CD20, MUM1, and Ki67 positivity. Conclusions: PCL was difficult to diagnose due to nonspecific clinical manifestations. Immunostaining helped to diagnose more specifically and determine the treatment plan. Histologic finding from PCL was mostly Diffuse Large B-cell lymphoma (DLBCL) and the prognosis was poor. Therefore, identifying the subtype of DLBCL was important for prognostic.

Left-sided primary cardiac lymphoma diagnosed by needle biopsy with total endoscopic anterolateral mini-thoracotomy: a case report

Abstract Background Primary cardiac lymphoma (PCL) is an extremely rare tumour that typically affects the right heart chamber. It is a life-threatening tumour presenting with rapid growth; therefore, early diagnosis and treatment are crucial for improving the prognosis of patients with PCL. Case summary An 81-year-old female with a history of dermatomyositis and interstitial pneumonia was referred to the cardiology department for cardiomegaly detected on chest radiography and computed tomography (CT). She experienced shortness of breath on exertion. Electrocardiography revealed negative T-waves in various leads. Transthoracic and transoesophageal echocardiography revealed a large mass on the epicardial free wall of the left atrium and ventricle. Coronary CT angiography showed feeding vessels from the left circumflex artery and the posterolateral branch of the right coronary artery. Positron emission tomography showed elevated mass uptake and no systemic metastasis. Needle biopsy with total endoscopic anterolateral mini-thoracotomy was performed. Histopathological examination revealed diffuse large B-cell lymphoma. She received systemic chemotherapy and achieved a complete metabolic response. Discussion Herein, we report an extremely rare case of PCL located on the left side of the heart. Owing to the location of the tumour, percutaneous or transcatheter biopsy could not be performed. Early diagnosis with needle biopsy via anterolateral mini-thoracotomy and systemic chemotherapy resulted in good outcomes.

Abstract 16992: Superior Vena Cava Syndrome in a Patient With Metastatic Primary Cardiac Diffuse Large B-cell Lymphoma - Case Report

Introduction: Primary cardiac tumors are uncommon, with an estimated incidence of 0.02%. Primary cardiac lymphomas (PCL), a subtype of non-Hodgkin lymphoma, account for about 10% of malignant and 1% of all primary cardiac tumors. Diffuse large B-cell lymphomas (DLBCLs) emerge as the predominant histological subtype within PCL cases. Case presentation: 54-year-old White male with no medical history presented with a few weeks of exertional dyspnea, nonproductive cough, and neck fullness. Exam was notable for tachycardia, jugular venous distension, bilateral upper extremity edema, and distended chest wall vasculature. Labs showed elevation of alkaline phosphatase and CRP. CT of the chest showed a right atrial mass extending into and obstructing the superior vena cava (SVC) and mediastinal lymphadenopathy (Panel A & B). Coronary CTA performed a few years earlier showed normal interatrial septum (Panel C) and coronary arteries. Transthoracic echo (TTE) confirmed a 7.6 x 5.0cm mass visualized in the interatrial septum extending to both atria. Cardiac MRI redemonstrated a large cardiac mass with malignant features suggestive of cardiac lymphoma vs angiosarcoma (Panel D-F). PET-CT showed enhanced fluorodeoxyglucose (FDG) uptake within cardiac mass, mediastinal, and intraabdominal lymph nodes (Panel G). Lymph node biopsy confirmed DLBCL. Patient underwent three cycles of EPOCH (etoposide phosphate, prednisone, vincristine sulfate, cyclophosphamide, doxorubicin hydrochloride) chemotherapy with excellent response. TTE following cycle 2 demonstrated decreased tumor size, and PET-CT after cycle 3 confirmed complete resolution of lymphadenopathy and minimal residual intracardiac FDG uptake. Conclusion: SVC syndrome can be a rare presentation of PCL. Multimodality imaging is important in cardiac mass characterization, diagnosis, treatment planning, and follow-up. Timely diagnosis and initiation of chemotherapy results in improved outcomes.

Primary cardiac lymphoma A comprehensive review

Objective: To summarize and analyze the clinical features, treatment and prognosis of patients with primary cardiac lymphoma. Method: We herein review the literature available in PubMed databases for recent five years. Then we analyze and summarize the literature data. Result: Among the 26 patients with primary cardiac lymphoma (PCL), the right atrium was most common site involved, and most tumors were single mass, the main symptoms were dyspnoea（65.4%），chest oppression (38.5%) and edema (19.2%). The elevations of lactate dehydrogenase (LDH) were observed, however the bone marrow aspiration or biopsy? Examinations were negative. Atrial fibrillation and atrioventricular block were also noted in some cases. The common expression of immunophenotyping were CD20, Ki-67, multiple myeloma oncogene 1 (MUM1), B-cell lymphoma/leukemia-6 (Bcl-6), CD10, and might be accompanied by expression of Bcl-2, c-myc, CD3 and CD5, while negative expression of CD30, anaplastic lymphoma kinase (ALK), Epstein-Barr virus encoded small RNA (EBER). The majority of patients in this cohort receiving R±CHOP chemotherapy (cyclophosphamide + pirarubicin + vincristine + prednisone with/without rituximab) were reported to have a poor prognosis with 1-year survival rate at 24.1% (9/26). Conclusions: PCL is very rare, with the variable clinical manifestations potentially leading to a delayed diagnosis.

C14 CASE REPORT: MULTIMODALITY IMAGE–CHALLENGE IN PRIMARY CARDIAC DIFFUSE LARGE B–CELL LYMPHOMA

Abstract A 76–year–old Caucasian woman presented to our hospital with progressive exertional fatigue and weight–loss over 2 months.Her past medical history revealed a recent local–hospital admission because of atrioventricular third–degree block, requiring permanent pacemaker implan­tation. At presentation her cardiovascular exam was normal.Laboratory studies revealed elevated LDH and PCR; electrolytes, cardiac biomarkers and complete blood count were normal. ECG demonstrated AF and ventricular stimulation.Transtoracich echocardiography showed LV hypertrophy in the basal segments, with normal ejection fraction and a reduced LV–GLS with a relative apical sparing pattern.There was a large, well–circumscribed mass arising from the right atrium, with a contextual moderate pericardial effusion (Fig1).Vasculitic, thrombophilic and infectious work–up were negative.The patient was immunocompetent. CMR was proposed but could not be performed due to patient claustrophobia. ECG gated cardiac CT was carried out for a better differential diagnosis of the mass, as well as to look for possible embolization or primary tumours, in the hypothesis of cardiac metastases. A CT total body showed a large ill–defined mass invading the interatrial and interventricular septum with projections into the left ventricle. Not enlarged lymph nodes were detected with neither hepatosplenomegaly nor abdominal lymphadenopathy. A total body 18FDG PET revealed significant tracer uptake in the heart and pericardium without extracardiac uptake, consistent with a malignant cardiac mass (Fig2).LV endomyocardial biopsy was attempted: pathology revealed large B–cell lymphoma expressing CD20, Bcl–2, Bcl–6, Mum–1, C–myc and with a high Ki67 proliferation rate. The patient underwent six cycles of chemotherapy(R–POCH).Echocardiography imaging after the second cycle showed significant reduction in myocardial wall thickness and concomitant improvement in LV–GLS. Repeated PET–CT scans 6 months post–chemotherapy showed complete remission with no evidence of FDG–avid lymphoma (Fig3). Primary cardiac lymphoma (PCL) is an extranodal non–Hodgkin’s B–cell lymphoma exclusively located in the heart or pericardium, accounting for 1–2% of primary cardiac tumours. PCL often presents with non–specific clinical symptoms making the diagnosis challenging. Multimodality approach and myocardial biopsy are essential in the diagnostic work–up. Early diagnosis and treatment are crucial since a good response to chemotherapy is expected.

P281 CARDIAC RICHTER‘S SYNDROME: A RARE CASE OF CHRONIC LYMPHOCYTIC LEUKAEMIA TRANSFORMATION

Abstract A 71–year–old woman with chronic lymphocytic leukaemia (CLL) presented to our Cardiology clinic complaining of recent–onset chest pain and dysphonia. Physical examination and ECG were normal. A blood test revealed high levels of high–sensitivity troponin T (373 ng/L) and of C–reactive protein (7 mg/dL); blood counts and differential were normal. Transthoracic echocardiography showed a hyperechogenic appearance of the interatrial septum, the interventricular septum and the right ventricular free wall, as well as a mild circumferential pericardial effusion. Cardiac magnetic resonance imaging revealed multiple intracardiac masses involving both ventricles and the interatrial septum, with evidence of oedema and fibrosis. A chest computed tomography showed the presence of multiple cervical, pulmonary, and mediastinal masses, one of which was biopsied through video–thoracoscopy. At the histological examination, the mass mainly consisted of large pleomorphic peripheral CD10–, CD20+/–, CD79a+, PAX5+, BCL2+, BCL6–, c–MYC+ B cells, with high proliferative activity (Ki–67/MIB1+ 80%). A diagnosis of CLL transformation into diffuse large B–cell lymphoma (DLBCL), also known as Richter’s syndrome (RS), was established (Figure). Despite indication for specific haematological chemotherapy, the patient rapidly deteriorated and died few days after the diagnosis. RS is a rare evolution of CLL and of small lymphocytic lymphoma, affecting 2–8% of such patients (transformation <1%/ year), yet is burdened with very poor prognosis (median overall survival 8–16 months). Extra–nodal RS is uncommon, and cardiac involvement has been reported in only 3 cases in the literature (in none with the panel of techniques here used). Despite its rarity, CLL patients with rapidly worsening cardiac symptoms should be suspected of RS transformation involving the heart. The main differential diagnosis of cardiac RS is primary cardiac lymphoma. The characterization of cardiac masses relies on multimodality imaging, but histologic analysis is required to reach a definite diagnosis, which is essential to guide subsequent therapeutic choices.

Tendon Cord Rupture of Tricuspid Valve During Chemotherapy for Primary Cardiac Diffuse Large B-Cell Lymphoma.

Tendon Cord Rupture of Tricuspid Valve During Chemotherapy for Primary Cardiac Diffuse Large B-Cell Lymphoma.

PO-05-126 A SHOCKING TURN OF EVENTS: PRIMARY CARDIAC LYMPHOMA AS A RARE CAUSE FOR SUDDEN CARDIAC DEATH

Primary cardiac tumors are rare causes of ventricular arrythmias. This presentation of primary cardiac lymphoma highlights the importance of multimodality cardiac imaging for patients who suffer sudden cardiac arrest. N/A N/A A previously healthy 34 year-old woman suffered an out-of-hospital cardiac arrest. She received bystander CPR and multiple shocks in the field for VT and VF. ECG obtained on arrival showed salvos of monomorphic VT with a LBBB morphology as well as polymorphic VT. Post-arrest ECG demonstrated dynamic changes with evidence of a prolonged QT interval and Brugada type 1 pattern. After stabilization with metoprolol and intravenous lidocaine, cardiac MRI showed an infiltrating mass along the epicardial surface of the right ventricular outflow tract (RVOT). A cardiac CT was subsequently performed for surgical biopsy planning. Biopsy was obtained via hemi-sternotomy; tissue histopathology of the RVOT mass revealed diffuse large B-cell lymphoma. PET and CT scans indicated no evidence of metastatic disease. The patient was diagnosed with primary cardiac lymphoma, and underwent inpatient chemotherapy treatment. A subcutaneous ICD was implanted prior to discharge for secondary prevention. Key images are displayed in Figure 1. This case demonstrates a rare cause of sudden cardiac arrest in a young patient with a new diagnosis of primary cardiac lymphoma. Primary cardiac lymphoma is a very rare disease that accounts for less than 1% of all primary cardiac malignancies, and less than 0.5% of all extra nodal lymphomas. The use of multimodality imaging after a cardiac arrest in this case led to a timely diagnosis and rapid initiation of life saving treatments.

Heart of the Matter: Reversal of Systolic Heart Failure and Long Term Remission in Primary Cardiac Lymphoma with Multi-Agent Chemotherapy with Continuous Intravenous Administration of Doxorubicin

Heart of the Matter: Reversal of Systolic Heart Failure and Long Term Remission in Primary Cardiac Lymphoma with Multi-Agent Chemotherapy with Continuous Intravenous Administration of Doxorubicin

Quantitative Characterization of Intertumor Heterogeneity of Primary Cardiac Diffuse Large B-Cell Lymphoma Using Cardiac Magnetic Resonance.

Quantitative Characterization of Intertumor Heterogeneity of Primary Cardiac Diffuse Large B-Cell Lymphoma Using Cardiac Magnetic Resonance.

Primary Cardiac Burkitt’s Lymphoma- A Deadlock

Burkitt’s lymphoma is a high-grade, extremely aggressive form of B-cell non Hodgkin lymphoma with a very fast replication time (tumour doubling time of around 25 hours). The three forms of Burkitt’s lymphoma are documented which include endemic, sporadic, and immunodeficiency associated. It is enormously rare with less than 30 cases reported till date. Primary cardiac lymphoma is a rare cardiac neoplasm. The most common type is diffuse large B-cell lymphoma. Diagnosis is usually late and with poor prognosis. Primary cardiac lymphoma is more common in immunodeficient patients. This is a case of primary cardiac Burkitt’s lymphoma which was diagnosed in a 58-year-old immunocompetent male. The patient’s two-dimensional echocardiography and plain Computed Tomography (CT) scan of the chest revealed presence of massive pericardial effusion with mild to moderate bilateral pleural effusion. Cardiac Positron Emission Tomography (PET) CT revealed a soft tissue mass at atrioventricular groove which indicated either pericardial lymphoma or inflammatory serositis. The patient was opted for surgery, but the mass was noted involving the free wall of right ventricle including the diaphragmatic surface which extended onto atrioventricular groove and base of right atrial appendage. Thus, a decision to not proceed further with surgical repair was made. The histopathological and cytogenetic findings confirmed the presence of Burkitt's lymphoma. Later, it was discovered that the patient died within one month of diagnosis.

Primary Cardiac Lymphoma: Case Series

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.

242 PRIMARY CARDIAC LYMPHOMA: A CASE REPORT

Abstract Introduction Primary cardiac lymphomas in immunocompetent patients are an uncommon malignancy, accounting for 1.3% of primary cardiac tumors, while metastases secondaries to extracardiac lymphomas are more common. Mean age at diagnosis is 63 years old. They mainly involve the right side of the heart, particularly right atrium. Symptoms of primary cardiac lymphoma are non-specific. Cardiac lymphoma typically follows an aggressive clinical course and the prognosis is poor, with more than 60% of patients dying within 2 months of diagnosis. Predictors of worse outcome are immune status, left ventricular involvement, arrhythmias and the presence of an extracardiac disease. Case report 48-year-old obese and hypertensive man, in therapy for OSAS. In February 2022 admission to the emergency department due to jugular pressure and mild exertion dyspnoea. Neck CT excluded inflammatory collections; echocardiography showed normal cavitary dimensions and biventricular systolic function, absence of major valvulopathies and circumferential pericardial effusion of n.d.d. with TD max 2.5 cm and partial collapse of the right atrium, for which started corticosteroid therapy. Chest CT showed mediastinal lymphadenopathy and persistence of pericardial effusion. In April, due to worsening dyspnoea, cardiac CT demonstated a solid mass at the passage from the superior vena cava to the right atrium (50×37×67 mm), leading to compression-infiltration phenomena on the superior vena cava, and thrombosis of the right mammary vein. Cardiac MR confirmed a voluminous expansive-infiltrative lesion, with an irregular lobular profile, projecting into the superior vena cava and extended to the right atrium. This mass appears very iperintensive in T2 stir sequences with an early contrast impregnation and uneven intralesional late enhancement (picture 1). With suspicion of lymphoma, after collegial discussion between thoracic surgeons, cardiologists, interventional radiologists and cardiac surgeons, a right transjugular radio- and echo-guided biopsy was performed to complete diagnostic iter; biopsy was positive for peripheral B lymphocyte-derived large-cell diffuse lymphoma with large necrotic and subnecrotic areas. Chemotherapy showed initial benefit,with a clear reduction in size of the cardiac mass and mediastinal lymphnodes at 2 months follow up, with regression of dyspnoea. Conclusions In our patient, despite an early lymphoma onset and a typical poor prognosis compared to literature, an early diagnostic-therapeutic iter, an immunocompetent status, the absence of extracardiac disease and a preserved left ventricular ejection fraction, ensured an immediate clinical benefit with an improvement of his quality of life.

Possibilities of early diagnosis of primary cardiac lymphoma. A clinical case

Tumor lesions of the heart remain an urgent problem due to high mortality. The complexity of early diagnosis of primary cardialymphoma is associated with the non-specificity of symptoms which often leads to the delayed verification of the diagnosis, commonly at stage 4. This article presents a clinical case demonstrating the need for instrumental cardiac diagnostics at an early stage in the presence of non-specific symptoms, such as heart conduction system pathology, hydrothorax, and hydropericardium syndrome.

A Rare Case of Primary Cardiac T-cell Lymphoma in Immunocompetent Adult

Abstract Introduction/Objective Primary cardiac lymphoma (PCL) is an extranodal lymphoma involving only the heart and/or the pericardium. It is exceedingly rare, accounting for <2% of primary cardiac neoplasms in a surgical series and 0.05% of a >12,000 autopsies’ series, and is often associated with an immunocompromised state. Most PCL are of B- cell lineage with T-cell lymphoma constituting < 10%. Methods/Case Report We report a case of unexpected sudden death due to primary cardiac T-cell lymphoma in a 48-year-old immunocompetent man without significant medical history who presented in cardiopulmonary arrest following a two-week history of cold-like symptoms. Autopsy revealed an irregular, discontinuous tan plaque on the epicardial surface that extended over the right and left ventricles, and sectioning revealed that extension of the lesion throughout the subvalvular myocardium of the left ventricular wall, interventricular septum, and medial aspects of the right ventricle. The lesion had irregular areas of necrosis and hemorrhage. No lymphadenopathy or other visceral lesions were identified. Histologically, the myocardium had a regionally necrotic, angiocentric infiltrate of atypical, large, pleomorphic lymphoid cells with large, irregular nuclei, vesicular chromatin, and conspicuous nucleoli. Initial immunohistochemical work-up showed a profile of CD45+/CD20-/CD3+ suggesting a T-cell origin with CD8 predominance (CD4-/CD8+). Further work-up demonstrated a profile as CD2+/CD7+/CD25+/CD30+/EBV(EBER- ISH)+/Ki67(70-80%)/ and ALK-1-/BCL6-/CD5-/CD10-/CD56-, supporting extranodal NK/T-cell lymphoma, extranasal type with a cytotoxic T-cell subtype (perforin+/granzyme B+/TIA-1+/TCR Beta F1+). Results (if a Case Study enter NA) NA. Conclusion Primary cardiac lymphoma with cytotoxic T-cell subtype is extremely rare and fatal. This case is the third report over the past two decades, to our knowledge, of a primary cardiac T-cell lymphoma leading to acute heart failure and sudden death in an immunocompetent adult.