The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspects must be discussed further in light of recent advances in lymphoma research. We collected cardiac lymphoma case reports published from January 2009 to January 2019, collected statistics from each patient, and performed a systematic analysis. The epidemiological characteristics, clinical manifestations, treatments, responses, and survival of primary cardiac lymphoma (PCL) patients are described. We obtained 158 cases of heart lymphoma, of which 101 were defined as PCL. There were more male than female cases. Most cases were diffuse large B-cell lymphoma. Six cases of PCL in cardiac myxomas were described. Patients with arrhythmia had shorter progression-free survival compared those without (HR 0.334, 95% CI 0.112-0.999, log-rank P = 0.042). Surgery did not improve patients' long-term prognosis or reduce the risk of death within 1 month. These data suggest that central nervous system prophylaxis is necessary. The overall survival was longer than that in data from 1949 to 2009.
Keywords: Cardiac lymphoma; Central nervous system involvement; Lymphoma in myxoma.