P11.48.A Primary CNS lymphoma after CLIPPERS: a case series

Abstract

Abstract Background Few cases of primary CNS lymphoma(PCNSL) following a diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) are reported in the literature. Here we explore implications of this sequel for CLIPPERS follow-up and PCNSL outcome. Material and Methods A systematic review and meta-analysis of previously described cases in the MEDLINE database was performed in compliance with PRISMA guidelines. Clinical, radiological and pathological characteristics were summarized. Overall survival was estimated using the Kaplan-Meier method. Results In addition to a case from our institution, nine cases were previously described in the literature. Eight patients fulfilled diagnostic criteria for ‘definite’ CLIPPERS whereas two cases were diagnosed with ‘probable’ CLIPPERS. Male-to-female ratio was 9:1. Median interval from CLIPPERS to PCNSL diagnosis was 4.5 months. All patients had received immunosuppressive therapy including methylprednisone in all and azathioprine or cyclophosphamide in most cases. On MRI, appearance of a larger lesion (>3mm) with peripheral contrast enhancement was noted in the most cases upon PCNSLdiagnosis. Whereas Epstein-Barr virus was not detected in biopsy specimen upon CLIPPERS diagnosis, it was identified in the majority of samples upon PCNSL diagnosis. Treatmentfor PCNSL included high-dose methotrexate basedchemotherapy in most cases, however median overall survival was poor (5.5 months). Conclusion Interval from CLIPPERS to PCNSL diagnosis was short and may mandate close clinical and radiological follow-up, specifically within the first two years after CLIPPERS diagnosis. Characteristics of PCNSL following CLIPPERS resemble other immunodeficiency-related PCNSL. Outcome was inferior and may reflect late diagnosis or brainstem localization.