a 35-year - old native american without significant medical history was transferred from another facility for the evaluation of worsening shortness of breath , cough , and hemoptysis of over 2 months . he also had a history of on and off fever , and weight loss of about 20 pounds . there was no history of recent travel or contact with tuberculosis patients . on examination , laboratory examination revealed hemoglobin of 7.5 g / dl and white blood cell count of 7,900/mm ( neutrophils 93% ) . antinuclear cytoplasmic antibody ( anca ) panel and antinuclear antibody ( ana ) were negative . a computerized tomography ( ct ) of the chest showed right - sided pleural effusion with mediastinal lymphadenopathy measuring 3.22.2 cm , and a ground glass density with coarsening of interstitial markings in bilateral lower lobes ( fig . 1 ) . pleurocentesis revealed pinkish fluid with 1,2750/mm red blood cells , 400/mm white blood cells ( neutrophils 17% , monocytes 31% , and lymphocytes 45% ) , 1.3 g / dl protein , 80 iu / l lactate dehydrogenase , 115 mg / dl glucose , and ph of 7.49 without any malignant cells . serum protein and glucose were 6.6 g / dl and 180 g / dl , respectively . ct scan without contrast showing right pleural effusion and right hilar adenopathy with suggestion of node or mass with size of 3.22.2 cm . transthoracic echocardiogram ( tte ) was done which revealed a large left atrial mass measuring 8.152.2 cm attached to interatrial septum on the left , causing pulmonary venous hypertension with an elevated right ventricular systolic pressure ( rvsp ) . this was followed by a transesophageal echocardiogram ( tee ) that revealed a mass completely occupying the left atrium and protruding across the mitral valve into the left ventricle with a mild mitral regurgitation . ( a and b ) 2-d echo showing atrial mass originating from left side of the interatrial septum extending beyond mitral valve to left ventricle ( measuring 8.152.24 cm ) . at the time of initial presentation with shortness of breath and hemoptysis , our differential diagnosis was broad and included connective tissue diseases , tuberculosis , lung malignancy , wegener 's granulomatosis , and heart failure . ana , anca , and quantiferon tests were negative , ruling out sle , wegener 's granulomatosis , and tuberculosis , respectively . cardiothoracic surgery was consulted for surgical excision of the mass , and the patient underwent a limited excision of the mass along with mitral valve repair . biopsy of the mass revealed high grade undifferentiated pleomorphic neoplasm composed of atypical cells with hyperchromatic nuclei and scattered atypical mitotic figures ( fig . a follow - up positron emission tomography ( pet ) scan showed residual disease activity in the pulmonary veins . patient was started on chemotherapy with ifosfamide and doxorubicin and was referred to a higher center for additional management . patient has received four cycles of chemotherapy so far and one dose of radiation treatment . at 9-month follow up , the majority of these tumors are benign , with the malignant tumors accounting for only about 25% of primary cardiac tumors ( 1 ) . cardiac sarcomas have been classified into angiosarcoma , fibrosarcoma , rhabdomyosarcoma , leiomyosarcoma , liposarcoma , pleomorphic malignant fibrious histiocytoma / pleomorphic undifferentiated sarcoma , and synovial sarcoma based on the classification proposed by who in 2004 ( 2 ) . among these subtypes , angiosarcomas and pleomorphic pcs may arise at any age , with a reported mean age of 41 years ( 6 ) . although , clinical presentation varies with the site of involvement , symptoms of congestive heart failure are the most common manifestation ( 4 ) . about a third of these tumors have evidence of systemic metastasis at diagnosis ( 7 ) . pcs located in the left atrium can cause obstruction of the mitral valve and can present with symptoms of mitral stenosis including dyspnea , cough , hemoptysis , and fatigue on exertion ( 8) . our patient presented with symptoms suggestive of mitral stenosis and constitutional symptoms of fever , weight loss , and fatigue , which have been reported previously , particularly with atrial myxomas ( 9 ) . undifferentiated pleomorphic sarcoma , as in our case , is a high grade malignant tumor characterized histologically by heterogeneous cellularity , pleomorphic appearance , high mitotic activity , and areas of necrosis . in some cases , such tumors comprise of small embryonal looking cells with no further recognizable differentiation ( 10 ) . immunohistochemical staining is crucial in these cases with sarcomas being negative markers for epithelial , neural , or endothelial elements ( 2 ) . in our case , . however , absence of staining for s100 , cytokeratin , myogenic , and myod1 made neural , lipomatous , epithelial tumor or rhabdomyosarcoma unlikely . the average duration of symptoms prior to diagnosis has been reported to be about 5 months ( 3 ) . the electrocardiogram findings are non - specific and include conduction deficits , low voltage , t - wave changes , and rhythm abnormalities . a chest x - ray may be normal or show cardiomegaly ( 3 ) . tee has been shown to be superior to a tte in identifying the presence and site of origin of primary cardiac tumors ( 11 ) . although it might be difficult to differentiate benign cardiac tumors from malignant ones based on the echocardiographic findings alone , it has been suggested that the presence of non - septal origin , broad attachment on the wall , extension into pulmonary vein , semisolid consistency , and multiple masses can predict the likelihood of a left atrial mass being a malignant ( 12 ) . a chest ct and mri can be helpful in the better delineation of anatomy for surgical planning as well as for evaluation of tumor extension ( 3 , 13 ) . prognosis of pcs remains poor with a reported median survival of 511 months ( 3 , 14 ) . the most important factor dictating prognosis is the ability to achieve a complete surgical resection of the tumor . it has been observed that the median survival increases to 1227 months with complete resection compared to 410 months where complete resection is not possible ( 1521 ) . hence , resection of tumor is indicated in all cases where possible and has an added benefit of effective palliation of symptoms ( 22 ) . however because of close proximity to vital structures , it is not always possible to achieve a complete surgical resection . reported a 97% reduction in the tumor size after starting oral etoposide in an undifferentiated pcs in a 9-year - old girl . chemotherapy has been tried , most commonly with doxorubicin and/or ifosfamide containing regimens , with unclear survival benefit . however these studies have been limited by small sample size ( 12 , 15 , 19 , 20 ) . hence , additional studies are warranted to identify the role of chemotherapy in these patients . orthotropic cardiac transplantation has been tried but the experience is limited ( 24 ) . in conclusion , pcs are rare malignant tumors of the heart with poor prognosis . left atrial tumors , patients present with signs and symptoms of left - sided heart failure . surgical resection is the mainstay of treatment , and the role of chemotherapy and radiotherapy remains unclear . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .
primary cardiac sarcomas are rare malignant tumors of the heart . 
 clinical features depend on the site of tumor and vary from symptoms of congestive heart failure to thromboembolism and arrhythmias . 
 echocardiography is helpful but definitive diagnosis is established by histopathology . 
 surgical resection is the mainstay of treatment , and the role of chemotherapy and radiotherapy is unclear . 
 we report a case of primary cardiac sarcoma which presented with signs and symptoms of acute left - sided heart failure .