Primary cardiac lymphomas are rare lesions in children with acquired immunodeficiency syndrome (AIDS). Most of them are high-grade Burkitt's or Burkitt-like lymphomas. They usually present with congestive cardiac failure, pericardial effusion or tamponade, arrhythmias, with predominant systemic 'B' symptoms and often with widespread extranodal involvement. The clinical profile and operative and pathological findings of a 4-year-old boy with AIDS-associated Burkitt's lymphoma of the heart presenting with acute right heart failure and fatal secondary pulmonary hypertension is reported.
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