nk / t - cell lymphomas ( nktcls ) represent an uncommon subtype of non - hodgkin 's lymphoma ( nhl ) , comprising only 5% of nhl cases in western countries . nktcls generally have poor outcomes , with high rates of relapse and progression even in the setting of limited stage disease . at least 80% of nktcls are localized at presentation , most commonly to the nasal cavity with extension to the nasopharynx and paranasal sinuses . however , distant sites of involvement may be observed , most commonly involving the skin , liver , lung , gastrointestinal tract , or testis . in cases of extranodal nktcls , we report a rare case of a patient with extranodal nktcl presenting with primary cardiac involvement . a 54 year old caucasian male with a history of polysubstance abuse and stage ia squamous cell lung carcinoma resected 2 years previously , presented to clinic with a 3 month history of progressive chest pain and dyspnea on exertion . computerized tomography ( ct ) imaging of the chest showed a 7.83.8 cm right atrial soft tissue mass infiltrating the lateral wall of the right atrium with associated pericardial effusion , and a 5 cm pericardiophrenic mass , both new when compared to ct imaging performed 6 months previously ( figure 1 ) . transthoracic echocardiography showed a large irregular , mural right atrial mass in the area of the tricuspid annulus with extensive infiltration of both ventricles and the posterior wall of the left atrium . in addition , he was noted to have a pericardial effusion with early tamponade physiology . a percutaneous core needle biopsy of the pericardiophrenic mass was performed ( figure 2 ) . hematoxylin and eosin stained sections of the mediastinal mass revealed a dense infiltrate of primarily small lymphocytes with irregular nuclear contours in a background of focal fibrosis and scattered histiocytes . immunohistochemistry and in situ hybridization demonstrated that the neoplastic cells co - expressed cd3 , cd56 , epstein - barr virus encoded rna , granzyme - b , and tia-1 . the final pathologic diagnosis was extranodal nk / t - cell lymphoma , nasal - type . 
 figure 1computerized tomography imaging confirms a large left pericardiac mass ( top right arrow ) and infiltrative mass arising from the right atrium ( left arrow ) . computerized tomography imaging confirms a large left pericardiac mass ( top right arrow ) and infiltrative mass arising from the right atrium ( left arrow ) . figure 2extranodal nk / t - cell lymphoma , nasal type . hematoxylin and eosin stained sections of the mediastinal mass reveals a dense infiltrate of primarily small lymphocytes with irregular nuclear contours ( a ) and immunohistochemistry shows the neoplastic cell co - express cd3 ( b ) and cd56 ( c ) . almost all of the neoplastic cells contained ebv - encoded rna ( eber ) when assessed by in situ hybridization . hematoxylin and eosin stained sections of the mediastinal mass reveals a dense infiltrate of primarily small lymphocytes with irregular nuclear contours ( a ) and immunohistochemistry shows the neoplastic cell co - express cd3 ( b ) and cd56 ( c ) . almost all of the neoplastic cells contained ebv - encoded rna ( eber ) when assessed by in situ hybridization . a unilateral bone marrow biopsy did not show any morphologic evidence of involvement by lymphoma . flow cytometry on the bone marrow biopsy was negative and immunostaining for eber was negative . retrospective review of his non - small cell lung cancer biopsy confirmed squamous carcinoma with no atypical findings , and eber immunostaining was negative . his blood counts at diagnosis were normal , including a normal ldh of 160 u / l . nasal endoscopy with biopsies and ct / pet imaging were recommended in order to complete the staging evaluation . multiple attempts were made to schedule and perform these procedures , but the patient was unwilling and/or unable to comply due to his chaotic psychosocial circumstances and his concerns about costs due to lack of medical insurance . although a more dose - intensive regimen would have been preferred , chop chemotherapy was administered for palliative treatment in this case due to issues with poor patient compliance and continued polysubstance abuse . two attempts had been made to administer the more dose - intensive smile ( steroids , methotrexate , ifosfamide , l - asaparaginase , and etoposide ) regimen as an inpatient , but the patient left within several hours of admission against medical advice . there were several intervening emergency department visits with tachyarrhythmias and cocaine intoxiciation . based on these limiting factors , the patient received 2 cycles of chop chemotherapy as an outpatient , but demonstrated disease progression on follow up ct imaging . the patient was then referred for palliative radiotherapy which he tolerated well with improved symptoms of chest pain and dyspnea . he developed acute abdominal pain approximately 1 week after completing radiotherapy , and repeat ct imaging showing a new confluent soft tissue mass encasing the duodenum , pancreatic head , and inferior vena cava . although improved outcomes have been reported with combined chemoradiation for treatment of limited stage nasal nk / t - cell lymphoma , outcomes remain poor for disseminated disease and extranasal nktcls . given the rarity of primary cardiac lymphomas , most clinical management decisions of this entity are extrapolated from experience with other aggressive lymphoma subtypes presenting with advanced stage disease and extranodal involvement . chemotherapy resistance of nktcls may be related to high rates of expression of p - glycoprotein , which is the product of the multidrug resistance ( mdr ) 1 gene . high - intensive chemotherapy regimens combined with radiotherapy may be a rationale means for bypassing the innate mdr of nktcls , with some limited data to support improved outcomes with such an approach . more recent reports have focused on combining l - asparaginase with chemotherapy agents known to be independent of mdr . for example , outcomes with the smile regimen , have reported rates of response exceeding 50% in a phase i study of relapsed , refractory nktcls . these promising results have led to on ongoing phase ii study of smile chemotherapy in asia . although prognosis remains poor in disseminated nktcls , prompt diagnosis and possible referral to a specialty center with experience in treatment of nktcls may offer patients the best opportunity for longer - term disease outcomes .
primary cardiac lymphoma is extremely uncommon . 
 we report a case of a 54 year old caucasian male with a history of non - small cell lung cancer treated by surgical resection who presented with chest pain and dyspnea on exertion . computerized tomography ( ct ) 
 imaging confirmed a 7.83.8 cm right atrial soft tissue mass infiltrating the lateral wall of the right atrium , and a 5 cm pericardiophrenic mass . 
 echocardiography confirmed a moderate pericardial effusion without tamponade physiology . 
 percutaneous biopsy of the pericardiophrenic mass revealed pathologic features diagnostic of nk / t - cell lymphoma . 
 he received chop chemotherapy with some improvement in symptoms , but experienced radiographic progression after 2 cycles . 
 he received palliative involved field radiotherapy but developed new sites of progressive disease within the abdomen and died shortly after completing radiotherapy . 
 nk / t - cell lymphomas are aggressive tumors that may present with unusual extranodal disease sites . 
 prompt diagnosis with consideration for referral to a specialty center with experience in treatment of these rare tumors may offer the greatest potential for improving treatment outcomes .