Abstract

ABSTRACT Aim: The incidence of primary central nervous system lymphoma (PCNSL) has been reported to increase in some countries while rates remained stable in others. Likewise, treatment of PCNSL patients has improved over the years with reported median overall survival (OS) rates of 30–60 months in clinical trials. The objective of this study was to analyze the contemporary incidence and survival of PCNSL patients at the population-level. Methods: Patients were identified by chart review of all cases with a recorded diagnosis of lymphoma within the central nervous system from 2005-2010 by the Austrian Brain Tumor Registry. By data linkage with the National Cancer Registry, all cases with evidence of a prior systemic lymphoma were excluded. Population data and last follow up (12/31/2012) were provided by Statistics Austria. Survival was analyzed using the Kaplan-Meier method. Results: From 2005–2010, there were 201 cases with a newly diagnosed, histologically confirmed PCNSL in Austria. The age-standardized incidence rate referred to 0.3 per 1,000,000 person-years (95% CI 0.2-0.4). Median age at diagnosis was 67ys (range 14-84ys) The median OS of all patients was 11.0 months. OS was 33.3% at 3 years and 21.1% at 5 years, respectively. While there were no significant differences in survival with regard to gender (p = 0.62; log-rank test) or tertiary care centers (p = 0.28; log-rank test), age was a major prognostic factor (median OS 44.0 months in patients aged 65 years of age). Conclusions: The incidence of PCNSL is low and patient median survival times at the population-level are considerably lower than those reported in clinical trials. Outcome is particularly poor in elderly patients. However, long-term survival beyond 5 years is achieved in approximately one fifth of patients. More research, including prospective and multicentric clinical trials, may help to optimize patient outcome in PCNSL. Disclosure: All authors have declared no conflicts of interest.

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