PRIMARY CARDIAC LYMPHOMA IN IMMUNOCOMPETENT PATIENTS - DIAGNOSTIC ANDTHERAPEUTIC MANAGEMENT

BACKGROUND, Primary cardiac lymphoma (PCL) is extremely rare in immuno competent patients. Different definition criteria have been employed i n published series. Prognosis is poor due to diagnostic delay and rele vance of the site of disease. METHODS, Two cases observed at the study institution are reported, with a review of 48 cases published in the literature from 1980 to 1996. Only patients with lymphoma confined to the heart and/or pericardium and those with a single and asymptomatic extracardiac site were considered for analysis. RESULTS, Eight patient s had minimal extracardiac disease. The most common presentation was u nresponsive heart failure. Electrocardiography findings were not speci fic. PCL usually arose in the right chambers as a mass, with or withou t pericardial effusion (> 80%). Chest X-rays, transthoracic echocardio graphy, and computed tomography scans are standard in diagnostic worku p, but transesophageal echocardiography (TEE) and magnetic resonance i maging (MRI) showed a sensitivity > 90%. Cytology of pericardial effus ion was diagnostic in 67% of cases. Thoracotomy was diagnostic in all cases, whereas less invasive procedures had high false-negative rates. Gross resection has no role. Early anthracycline-containing chemother apy appears to improve survival, whereas the role of radiotherapy has not yet been defined. CONCLUSIONS, The diagnosis of PCL should be cons idered in patients with a cardiac mass and/or unexplained refractory p ericardial effusion. Adequate diagnostic workup, including TEE and MRI , allows confirmation of the early suspicion of PCL. in the absence of a diagnostic cytology, an open biopsy may be indicated to avoid treat ment delay. There is no evidence that PCL should be treated differentl y from other bulky aggressive lymphomas arising at other anatomic site s. (C) 1997 American Cancer Society.