Abstract

e13020 Background: PCNSL is a rare variant of diffuse large B cell lymphoma. Optimum treatment strategies are yet to be defined. We report the Irish experience. Methods: We examined the Irish National Cancer Registry to assess the incidence of PCNSL. Patients diagnosed between 2001 and 2011 were cross referenced and identified from medical oncology, neuropathology and pharmacy database in the 3 cancer centres. Progression-free survival (PFS) and overall survival (OS) were estimated via Kaplan-Meier methods, and Cox proportional hazard model was used to identify risk factors. Results: 74 patients were treated in the 3 centres over the time period, representing 57% of all cases diagnosed in Ireland (ranging from 12.5% in 2001 to over 90% from 2008 onwards). 39 (52.7%) were male, with a median age of 60 years (range 17 – 78). 64% had an ECOG PS of 0 – 1 at presentation. Involvement of the deep structures occurred in 37.5% (n=24), with 29.7% (n = 19) having multifocal disease. 20.3% presented with hemiparesis with 18.6% presenting with symptoms suggestive of raised intracranial pressure. 80% were treated with the DeAngelis regimen and 14% with the Ferreri regimen. 35.7% received whole brain radiotherapy. The median PFS was 22.4 months, (95% CI, 14.2 – 32.1), and median OS was 33.3 months, (95% CI, 22.3 – 68.8). ECOG PS and presence of hemiparesis at diagnosis were independent poor prognostic factors for PFS but not for OS. Age, tumour site, multi-focality, chemotherapy regimen and delivery of radiotherapy did not significantly affect PFS or OS. Conclusions: Our data compares favourably with published series. Increased understanding of the molecular biology of PCNSL is urgently needed in order to define optimum therapies. PCNSL should be treated in specialist centres to facilitate increased casemix, enrolment in clinical trials and banking of tumor tissue.

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