Primary cardiac lymphoma

BACKGROUND: Primary cardiac lymphoma (PCL) represents a rare subset of non-Hodgkin lymphoma, characterized by poor outcomes. The authors aimed to construct a framework of known clinical presentations, diagnostic features, disease complications, treatment, and outcomes to improve prognostication. METHODS: Individual patient data were obtained from defined cases of PCL (1949-2009) and systematically analyzed. RESULTS: The authors report results of a review of 197 cases of PCL, with half of all cases reported since 1995. Survival was affected by 4 factors: immune status, left ventricular involvement, presence of extra-cardiac disease, and arrhythmia. Median overall survival (OS) for immunocompromised and immunocompetent was 3.5 months (m) and not reached, respectively (HR 0.29, 95% CI, 0.13-0.68; P = .004). LV involvement was uncommon (26%) and associated with an OS of only 1 m, whereas patients free of LV involvement had a median OS of 22 m (HR 0.28, 95% CI, 0.12-0.64; P = .002). Patients with extracardiac disease had shorter median OS compared with those without (6 m vs 22 m, HR 0.49, 95% CI, 0.26-0.91; P = .02). Those patients with an arrhythmia of any type had a median OS that was not reached (n = 55), whereas those without rhythm disturbances (n = 41) had median OS of 6 m (HR 0.51, 95% CI, 0.29-0.91; P = .024). Overall response rate to therapy was 84%, with long-term OS over 40%. CONCLUSIONS: The current study presents the largest analysis of PCL to date. The data demonstrate that PCL is now more frequently diagnosed premortem and appears to have reasonable response rates. Lack of LV involvement and the presence of arrhythmias are associated with improved survival.