Diagnosis Of Primary Cardiac Lymphoma Research Articles

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.

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.

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.

Primary cardiac lymphoma.

This study aimed to understand the population-level treatment modalities and to evaluate the survival benefits of surgical resection in primary cardiac lymphoma. We queried the Surveillance, Epidemiology, and End Results Program database, which covers 35% of the US population. Patients with a histologic diagnosis of primary cardiac lymphoma from 1973 to 2015 were included. Multivariable accelerated failure time regression was performed to evaluate the associations between clinical factors and overall survival. A total of 184 patients were identified. The median age was 68years, 80% were White, and 46% were women. Diffuse large B-cell lymphoma (80%) was the most common histology, and the majority (65%) was low-stage lymphoma (Ann Arbor stage I or II). Median survival was 2.2years. Seventy-three percent of patients received chemotherapy. Only 10% of patients received local resection or debulking. Multivariable analysis demonstrated that local resection or debulking was not independently associated with overall survival (adjusted hazard ratio, 0.67; 95% confidence interval, 0.30-1.48; P=.32). Instead, chemotherapy (adjusted hazard ratio, 0.4; 95% confidence interval, 0.23-0.69; P<.001) was independently associated with improved survival, whereas increasing age (adjusted hazard ratio of 5-year increment, 1.13; 95% confidence interval, 1.04-1.22; P <.001) and advanced stage (adjusted hazard ratio, 2.18; 95% confidence interval, 1.33-3.56; P<.001) were independently associated with worse survival. Surgical resection was not independently associated with survival in patients with primary cardiac lymphoma. Chemotherapy was the predominant treatment option and associated with improved survival, whereas increasing age and advanced stage were independently associated with worse outcomes.

Multimodal imaging evaluation of a primary cardiac lymphoma in an immunocompetent patient

Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma that involves only the heart and/or pericardium. It is fatal in the absence of a prompt diagnosis and adequate therapy. Here, we report an immunocompetent 55‐year‐old Chinese man with palpitations and occasional chest distress for 1 month. Multimodal imaging work‐up showed a tumor infiltrated into the myocardium, which greatly contribute to a timely pathology diagnosis of PCL.

Cardiac Lymphoma Revealed by Atrial Mass

Primary cardiac lymphoma (PCL) represents a very exceptional clinical entity whose treatment is based on chemotherapy. In this case report, the authors highlight the key role of cardiac surgery in the diagnosis of PCL in a 66-year-old female patient. She was given chemotherapy and so far was on remission.Cardiovasc. j. 2018; 11(1): 86-88

The First Case of Primary Cardiac Lymphoma, Diffuse Large B-Cell Type, Successfully Treated with EPOCH-R

Introduction:Primary cardiac lymphomas are rare non-Hodgkin lymphomas and account for less than 1% of intrinsic cardiac tumors and extranodal Non-Hodgkin lymphomas. Patients can present with a variety of manifestations including heart failure, chest pain, dyspnea, arrhythmias, and tamponade. Presently, there are no formal established guidelines for treatment of primary cardiac lymphomas.Presentation:This is a case of a 72-year-old white man with past medical history significant for paroxysmal atrial fibrillation and known pulmonary, renal, and cutaneous sarcoidosis who presented with palpitations, dyspnea on exertion and lightheadedness for two-weeks. He was evaluated by his cardiologist who recommended an elective atrial fibrillation radiofrequency ablation (RFA). Prior to the planned procedure a transesophageal echocardiogram was performed which revealed a large tissue density within the pericardial space, adjacent to the free wall of the right ventricle and right atrium, and invading the myocardium with extension through the interatrial septum (Figure 1). The RFA was aborted and the patient was admitted for further evaluation of the new cardiac mass, initially suspected to be cardiac sarcoidosis.Subsequent work up included a cardiac MRI, which displayed a dense 7.5 cm by 3.5 cm mass involving the right ventricle and right atrium, wrapping around the great vessels. Right heart cardiac catheterization with endomyocardial biopsy was performed which revealed clusters of large atypical B lymphoid cells with the following immunophenotype: CD30+, CD79a+, Pax5+, Alk 1-, CD138-, CD 10-, Bcl 2-, Bcl 6-, CD45-, focally CD20+, Ki67: 80-90% (Figure 2). These findings were consistent with diffuse large B cell lymphoma (DLBCL). His PET-CT demonstrated absence of extracardiac lesions, confirming the diagnosis of primary cardiac lymphoma (PCL) (Figure 3).The patient began treatment with a EPOCH-R (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) modified chemotherapy based regimen which consisted of a pre-phase treatment with dexamethasone 12 mg once daily for four days, a cumulative dose of cyclophosphamide 600 mg/m2 over five days and etoposide 50 mg/m2 for three days. Doses of cyclophosphamide and etoposide were initiated at lower doses and titrated upward to reach the target dose. This strategy was invoked based upon prior experiences and studies that suggest that a modest pre-phase cytoreduction reduces the risk of cardiac perforation. Rituximab was given on day six. He was also given granulocyte colony stimulating factor (GCSF).Our patient was observed closely in the cardiac intensive care unit throughout the duration of chemotherapy due to the high risk of fatal arrhythmia, tamponade, perforation and heart failure. Interim restaging, confirmed complete remission of the intracardiac mass. His post chemotherapy TTE demonstrated a slight reduction in ejection fraction (Figure 1). He received a total of three cycles of EPOCH-R and remained hemodynamically stable within the hospital. No further chemotherapy was given due to a decline in the patient's performance status.Conclusion:This is the first documented case of primary cardiac diffuse large B cell lymphoma successfully treated with EPOCH-R consisting of a modest pre-phase cytoreduction with steroids, cyclophosphamide and etoposide. The high area under the curve of doxorubicin in this setting is far less cardiotoxic. This regimen reduces the risk of cardiac perforation in patients with relatively normal ejection fractions. [Display omitted] [Display omitted] [Display omitted] DisclosuresO'Connor:Mundipharma: Membership on an entity's Board of Directors or advisory committees; Seattle Genetics: Research Funding; TG Therapeutics: Research Funding; Bristol Myers Squibb: Research Funding; Spectrum: Research Funding; Celgene: Research Funding; Bristol Myers Squibb: Research Funding; Celgene: Research Funding; TG Therapeutics: Research Funding.

Primary Cardiac Lymphoma Presenting as an Atypical Type of Hypertrophic Cardiomyopathy

Primary cardiac lymphoma (PCL) is a very rare malignancy although cardiac involvement with the disseminated disease is not uncommon. We present a case of a 43-year-old man with PCL that initially presented as marked thickening of all cardiac walls and was mistakenly diagnosed as an atypical type of hypertrophic cardiomyopathy. The diagnosis of PCL was made with a delay of 9 months after the initial presentation, when atypical lymphocytes staining positive for CD79a and CD20 were demonstrated in the rapidly growing mediastinal and neck mass. Anthracycline-based chemotherapy and anti-CD20 immunotherapy resulted in a remarkable reduction in cardiac wall thickness and mediastinal mass. The first lesson to be learnt from this case is that PCL can present as a diffuse infiltrative disease without a mass. The second lesson is that prompt exploratory thoracotomy should not be delayed when the diagnosis is elusive.

Linfoma cardíaco primário em cão

Lymphomas are malignant neoplasm characterized by proliferation of lymphocytes that originate primarily in lymphoid organ such as lymph nodes, liver, spleen and bone marrow. However the feature of continuous migration of lymphocytes in different organs, this tumor can develop in any organ. Although lymphoma is a very common hematopoietic neoplasm in dogs, cardiac location is rare. The diagnosis of primary cardiac lymphoma may be performed when there is involvement of the heart and / or the pericardium without evidence of involvement in other organs. In veterinary medicine there are few reports on the diagnosis, treatment and prognosis of cardiac lymphoma. Therefore, the purpose of this report is to describe a case of cardiac lymphoma in which the patient responded favorably to chemotherapy employee with disease-free interval of 19 months and highlight the importance of including this neoplasm in the differential diagnosis of diseases that affect the cardiovascular system.

Primary cardiac lymphoma mimicking infiltrative cardiomyopathy

Primary cardiac lymphoma is a rare malignancy which has been described as thickened myocardium due to the infiltration of atypical lymphocytes and accompanying intracardiac masses. Here, we report a case of a primary cardiac lymphoma without demonstrable intracardiac masses, mimicking infiltrative cardiomyopathy. A 40-year-old male presented with exertional dyspnoea and was diagnosed as having restrictive cardiomyopathy with severely decreased LV systolic function. Endomyocardial biopsy was performed and the diagnosis of primary cardiac lymphoma was confirmed. After appropriate chemotherapy, he recovered his systolic function fully.

Echocardiographic findings and clinical signs in dairy cows with primary cardiac lymphoma: 7 cases (2007–2010)

To describe echocardiographic and clinical findings in cattle with cardiac manifestation of lymphoma. Retrospective case series. 7 adult Holstein dairy cows with cardiac lymphoma. Medical and necropsy records of all cows that underwent transthoracic echocardiography from January 2007 through April 2010 because of clinical signs of cardiac diseases or bovine lymphoma were reviewed. The diagnosis of cardiac manifestation of lymphoma was confirmed by necropsy examination or cytologic evaluation of pericardial fluid obtained by pericardiocentesis. Most commonly, cows had clinical signs of right-sided congestive heart failure secondary to cardiac tamponade with moderate to severe anechoic pericardial effusion (5/7 cows). In 2 cows, tachycardia was the only clinical sign in relation to cardiac disease and a heterogenic mass was observed protruding into the right atrium. The use of transthoracic echocardiography aided in the diagnosis of primary cardiac lymphoma in cows. Transthoracic echocardiography may help to quantify the severity of pericardial effusion and to orient needle placement for pericardiocentesis to determine a final diagnosis. A mass within the right atrium was also suggestive of cardiac lymphoma and should be differentiated from mural bacterial endocarditis.

Primary cardiac lymphoma

Primary cardiac lymphoma (PCL) is a very rare disorder. Histologically, the majority of cases of PCL are diffuse B-cell lymphoma. PCL occurs more frequently in immunocompromised patients. Symptoms may vary according to the heart site involved. The most frequent cardiac clinical manifestations associated with PCL are pericardial effusion, heart failure, and atrioventricular block (AV-block). Diagnosis of PCL can be suggested by transesophageal echocardiography, computed tomography, and magnetic resonance imaging. However, cytologic examination of cardiac tumor or pericardial effusion is paramount for a definite diagnosis of this condition. Prognosis of PCL is poor with a median survival of 7 months after initial diagnosis. Newer modalities including immunotherapy with rituximab or auto stem cell transplantation are promising in the treatment of this lethal condition.

Coronary Sinus Obstruction by Primary Cardiac Lymphoma as a Cause of Dyspnea Due to Significant Diastolic Dysfunction and Elevated Filling Pressures

A 52-year-old woman presented with severe dyspnea of 2 weeks' duration. Echocardiography showed an enlarged left atrium but normal global left ventricular systolic function. However, a huge, irregularly shaped mass at the dilated coronary sinus that extended into the right atrium was noted. Mitral inflow showed restrictive physiology and the E/E' ratio was significantly elevated, suggesting elevated left ventricular filling pressures. Echocardiography-guided biopsy was performed, and a diagnosis of primary cardiac lymphoma (diffuse large B-cell type) was made. After the first cycle of chemotherapy, the patient's symptom was markedly improved. A follow-up echocardiogram showed complete removal of the mass and a change in left ventricular filling pattern from restrictive to relaxation abnormality with decreased E/E'. The present case demonstrates a rare cause of diastolic dysfunction due to coronary sinus obstruction by tumor infiltration. Diastolic dysfunction caused by coronary sinus obstruction was improved after the tumor was resolved by chemotherapy.

Extensive Primary Cardiac Lymphoma Diagnosed by Percutaneous Endomyocardial Biopsy

Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is rare in immunocompetent patients and represents 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. The clinical behavior is aggressive and the early symptoms are cardiac failure, syncope, arrhythmia, or pericardial effusion. Although echocardiography, computed tomography (CT) scan, magnetic resonance image (MRI) are the mainly used imaging techniques to detect cardiac tumors, pathologic examination is always required to confirm the diagnosis. Diagnosis of PCL is difficult due to non-specific clinical manifestations and requires invasive approach to get histopathologic evidence. While surgery with systemic chemotherapy or in combination with irradiation has been attempted, the only effective treatment is chemotherapy. However, the prognosis remains poor. We report on a 42-year-old woman who is diagnosed histopathologically as PCL by cardiac catheterization assisted percutaneous endomyocardial biopsy and treated successfully by anthracycline based chemotherapy.

A Case of Primary Cardiac Lymphoma: Analysis of the Role of Echocardiography in Early Diagnosis

Primary Cardiac Lymphoma (PCL) is defined as a non-Hodgkin's lymphoma involving only the heart and/or pericardium. Diagnosis of this rare disease is particularly difficult due to its nonspecific clinical manifestations. In this review the role of echocardiography in the early diagnosis of PCL is assessed, moreover we report an improvement in the outcome of PCL related to increased availability and utilization of echocardiography.

The diagnosis of primary cardiac lymphoma by right heart catheterization and biopsy using fluoroscopic and transthoracic echocardiographic guidance

We describe a case of primary cardiac lymphoma diagnosed by transvenous biopsy under fluoroscopic and transthoracic echocardiographic guidance. A 38-year-old man was admitted because of exertional dyspnea. ECG revealed complete atrioventricular block. Transthoracic echocardiography revealed a large mass attached to the interventricular septum and protruding into the right atrium. The patient underwent a right heart catheterization and a biopsy was taken from the mass using fluoroscopic and transthoracic echocardiographic guidance. Diagnosis of malignant lymphoma was established by the biopsy specimen. The use of transthoracic echo in conjunction with fluoroscopy may be useful for the diagnosis of intracardiac mass transvenously.

Primary Cardiac Lymphoma: Diagnosis by Transvenous Biopsy Under Transesophageal Echocardiographic Guidance

A 64-year-old woman presenting with dizziness and atrioventricular conduction disturbances was found to have a right atrial mass by two-dimensional transthoracic echocardiography. Transesophageal echocardiography allowed further delineation of the tumor and safe performance of transvenous biopsy, thereby obviating the need for surgery. Pathological examination of the biopsy specimen as well as the absence of extracardiac location established the diagnosis of primary cardiac lymphoma. (J Am Soc Echocardiogr 1998;11:89-91.)

The Applications of PCR to the Diagnosis of Primary Cardiac lymphoma

A 75-year-old man initially complained of pollakiuria and low abdominal pain, and died of massive bleeding from an exacerbated gastric ulcer. The diagnosis of primary cardiac lymphoma was made postmortem. The tumor involved only the epicardium and myocardium, which met the criteria of primary cardiac lymphoma as defined by the Armed Forces Institute of Pathology. The lymphoma consisted of large cells and expressed the B cell marker, CD20. Although chronic inflammation due to chronic renal failure was observed in the pericardium around the lymphoma, polymerase chain reaction (PCR) was conducted to detect monoclonality at the DNA level in lymphoma cells, which were shown to comprise a monoclonal population.

Primary Cardiac Lymphoma

An elderly man presented with pericardial tamponade: a diagnosis of primary cardiac lymphoma was made by a cytological examination of pericardial fluid. An excellent response to therapy has been observed.