primary cardiac lymphoma ( pcl ) , which makes up about 2% of all primary cardiac tumors , is extremely rare in immunocompetent hosts . the median age of patients with pcl is 64 years , and it occurs 3 times more frequently in women than in men . the symptoms at initial presentation are nonspecific and examples of symptoms include obstruction of blood flow , tumor invasion to adjacent organs , symptoms indicating tumor emboli , treatment - refractory heart failure , dyspnea , arrhythmia , superior vena cava syndrome , and stroke . imaging modalities such as echocardiography ( ecg ) , computed tomography ( ct ) , and magnetic resonance imaging ( mri ) are helpful , and pathologic findings by tissue biopsy or surgical resection are essential for a confirmative diagnosis . in this paper , we present a case of a 59-year - old man who presented with chest pain . a huge mass in his right atrium was detected , and diffuse large b - cell lymphoma was diagnosed through a percutaneous endomyocardial biopsy . he experienced a disease - free state of over 4 years of outpatient follow - up , after he received the systemic chemotherapy regimen of rituximab , cyclophosphamide , doxorubicin , vincristine , and prednisolone ( r - chop ) and additional post - chemotherapeutic radiation therapy . a 59-year - old man without a medical history visited a local clinic due to a 1-month history of chest pain . an ecg was performed , and a mass - like lesion was detected in the right atrium . his blood pressure was 121/79 mmhg , pulse rate was 76/min , respiratory rate was 18/min , and body temperature was 36.4. his heart sounds exhibited a slightly accelerated regular rhythm without a murmur . a fixed s2 split of his heart sounds was heard . his bowel sounds were normoactive , and there was no tenderness or rebound tenderness in the abdomen . the laboratory findings of the patient 's peripheral blood samples were as follows : white blood cell count , 8,500/l ; hemoglobin , 14.4 g / dl ; and platelets , 352,000/l . the results of serology were as follows : hbs ag / ab ( -/+ ) ; anti - hcv ( - ) ; and anti - hiv ( - ) . an initial ecg showed a first - degree atrioventricular block , a right axis deviation , and a suspicious right ventricular hypertrophy . a transthoracic ecg showed a 64 cm - non - movable ovoid mass with a broad fundus in the atrial and membranous septum of the right atrium . this mass nearly obstructed the inflow to the right ventricle , which resulted in reduced diastolic volume of the right ventricle . the ejection fraction of the left ventricle was 69% , and a moderate amount of pericardial effusion was present . a chest ct showed that the poorly enhanced tumor pressed on both the atria and part of the left ventricle and was accompanied by pericardial effusion . a cardiac mri showed a mass that invaded the left atrium and extended to the pericardial space and intra - atrial cavity ( fig . this mass encased the root of the aorta , the proximal part of the right coronary artery , and the left main coronary artery . it showed an iso - tense signal intensity on a t2-weighted image , a diffuse enhancement pattern on a contrast - weighted image , and an iso - tense or slightly higher signal intensity compared to that of cardiac muscle on a t1-weighted image . a cardiac catheter - guided endomyocardial biopsy was performed at 8 sites in the heart . the pathologic findings were consistent with diffuse large b - cell lymphoma , and the specimen was cd20-positive ( fig . tumor invasion of the malignant lymphoma at any area except the heart was not detected in a bone marrow examination or in the imaging studies . the patient received systemic chemotherapy of a rituximab , cyclophosphamide , adriamycin , vincristine , and prednisolone ( r - chop ) regimen every 3 weeks . the size of the mass was remarkably reduced after 1 cycle of r - chop , and further reduced after 6 cycles of chemotherapy ( fig . the patient received radiation therapy to the cardiac mass while in the pr state , and the total dose of radiation was 39.6 gy in 22 fractions . however , the size of the cardiac mass did not change despite the radiation therapy , and the disease state continued to be pr after the completion of the radiotherapy . the patient visited the emergency room due to chest pain 3 years after the completion of the radiation therapy . the cardiac imaging showed no change in the size of the cardiac tumor , whereas the findings of the coronary angiography showed total occlusion of the proximal part of the left anterior descending ( lad ) coronary artery . he received coronary artery bypass graft surgery due to an acute myocardial infarction of the lad territory . the disease state of the patient has been complete clinical remission over the 4 years since the initial diagnosis of pcl . to our knowledge , this is the first report of a case with long - term disease - free survival in a korean patient with pcl treated with systemic chemotherapy combined with post - chemotherapeutic radiation therapy . a summary of cases of pcl in korea is available elsewhere . in order to compare this case with previous reports of patients with pcl , we searched case reports from the database of the national library of medicine of the united states ( http://www.ncbi.nlm.nih.gov/pubmed/ ) according to the following criteria : ( 1 ) published from jan 1995 to dec 2009 , ( 2 ) treated with systemic chemotherapy ( except adjuvant chemotherapy ) , ( 3 ) long - term follow - up data ( 1 years ) was available , and ( 4 ) written in english . the analysis of the previous reports showed that the predominant gender was male ( 75.0% ) . the most common histologic subtype was diffuse large b - cell lymphoma , and the most frequent site of the tumor was the right atrium ( table 1 ) . the most frequent location of pcl reported in our results was different from that of myxoma , which is the most common primary cardiac tumor . the clinical presentation of pcl is nonspecific , and hence , an imaging study that is conducted because of a suspicion for pcl is essential to the initial diagnosis . there are a few primary cardiac malignancies that mimic pcl , such as angiosarcoma and rhabdomyosarcoma . angiosarcoma is usually located in the central region and shows an iso - tense signal intensity , which is consistent with central necrosis on t1-weighted images , whereas pcl shows a nodular mass without intratumoral necrosis of an iso - tense signal intensity on t1-weighted images . rhabdomyosarcoma that originates from the myocardium has no favorable location and frequently invades the valvular structure . an imaging study of rhabdomyosarcoma shows a huge mass with central necrosis and a characteristic inhomogeneous signal intensity on t1- and t2-weighted images . despite the few differential considerations of primary cardiac malignancies described above for example , pcl may have less enhanced central regions , which is consistent with necrosis . although fluorine-18 fluorodeoxyglucose ( f - fdg ) positron emission tomography ( pet)-ct is performed for the staging in many cases , the diagnostic sensitivity of a cardiac lesion is very low because the baseline fdg uptake of the heart is very high . therefore , ct and mri , rather than pet - ct , were performed in the evaluation of our patient . the standard treatment option of pcl is not well established , and various treatment modalities , such as surgical resection , systemic chemotherapy , radiation therapy , and combined treatments , have been attempted . the analysis of the 13 case reports , including the current case report , showed that all patients received anthracycline - containing combination chemotherapy with or without additional post - chemotherapeutic radiation therapy . although the number of patients enrolled in our analysis was too small to elicit statistical significance , our analysis of the case reports of pcl showed that the patients who were treated with a combined modality of systemic chemotherapy and additional post - chemotherapeutic radiation therapy had a better long - term progression - free survival ( pfs ) compared to those treated with systemic chemotherapy alone ( median pfs , 41.0 months vs. 12.0 months ) . furthermore , no recurrence of lymphoma was noted in the patients who received both systemic chemotherapy and additional post - chemotherapeutic radiation therapy . the patients who received rituximab - containing regimens had a longer pfs compared to those who received non - rituximab - containing regimens ( median pfs , 24.0 months vs. 12.0 months ) . however , 1 case of recurrence was noted among the patients who had received rituximab - containing regimens ( table 2 ) . our analysis of this small case series of pcl patients showed that the combined treatment of chemotherapy and post - chemotherapeutic radiation therapy decreased the risk of relapse . the cardiac mass of the patient in our case showed a residual lesion that was unchanged for a long time after the completion of the radiation therapy , which suggested the probability that non - tumorous fibrous tissue replaced the original tumor subsequent to the chemotherapy - induced tumor necrosis . although cases of cardiac perforation as an initial presentation have been reported [ 7 , 8 ] , a post - chemotherapeutic complication of a cardiac perforation has not been reported yet . a previous report showed that myocardial infarction developed in 4.5% of non - small cell lung cancer patients who had received previous radiation therapy , and another report of 415 patients with hodgkin 's disease   showed that coronary artery disease developed in 42 patients ( 10.4% ) , a median of 9 years after radiation therapy . other cardiac diseases such as valvular heart disease , pericardial disease , and cardiomyopathy have also been reported . although the patient described in this case report had no risk factors for coronary artery disease , an acute myocardial infarction developed 3 years after the completion of radiation therapy , and was successfully treated by coronary artery bypass graft surgery . although the addition of radiation therapy is thought to decrease the risk of relapse in the treatment of patients with pcl , a careful and thorough consideration of the potential complications of radiation therapy should be considered .
primary cardiac lymphoma ( pcl ) is a rare disease entity with only a few reported cases in korea . in this paper 
 , we report a case of pcl in a 59-year - old man presenting with chest pain . 
 diffuse large b - cell lymphoma was diagnosed through a cardiac catheterization - assisted percutaneous endomyocardial biopsy , and there was no evidence of extracardiac involvement of the lymphoma.the patient had a complete clinical response after systemic chemotherapy with a rituximab , cyclophosphamide , doxorubicin , vincristine , and prednisolone ( r - chop ) regimen and additional post - chemotherapeutic radiation therapy . 
 the patient experienced a long - term disease - free survival of over 4 years . 
 however , he received coronary artery bypass graft surgery due to an acute myocardial infarction that occurred 3 years after the completion of the radiation therapy . 
 although the addition of radiation therapy to the treatment is thought to decrease the risk of relapse in patients with pcl , a careful and thorough consideration of the potential complications of radiation therapy , particularly with respect to cardiac complications , should be considered .