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Primary CNS lymphoma (PCNSL) represents an extranodal variant of non-Hodgkin’s lymphoma (NHL) confined to the central nervous system (CNS) at diagnosis.1 Few cases of PCNSL following an initial diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), an inflammatory CNS condition with suspected autoimmune aetiology, have previously been described in the literature.2–4 In a recent study, 13 of 42 presumptive CLIPPERS cases were eventually diagnosed with various CLIPPERS mimics.3 NHL represented the largest subset of CLIPPERS mimics including two cases of PCNSL. Whether PCNSL represents a CLIPPERS mimic or complication remains subject of ongoing discussion. Some authors hypothesised CLIPPERS could be an early phase of CNS lymphomatoid granulomatosis (LYG) with subsequent Epstein-Barr virus (EBV)-associated neoplastic transformation into CNS lymphoma.5 Characteristics of PCNSL following CLIPPERS remain poorly defined. Here, we analysed 10 cases and explored implications for CLIPPERS management and PCNSL outcome.
A comprehensive review on PCNSL following CLIPPERS was independently conducted by two authors using the Medline database in compliance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in August 2020. ‘CLIPPERS’ was used as the primary search term. Boolean operator ‘and’ was applied together with the secondary search term ‘lymphoma’ to narrow results. A total of 433 articles underwent screening for eligibility. Only cases with a diagnosis of ‘probable’ or ‘definite’ CLIPPERS according to updated diagnostic criteria and a tissue-based diagnosis of PCNSL or high-grade LYG were included.4 Eight articles matched inclusion criteria and were selected …
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CS and LDK contributed equally.
Contributors SD, CS and LDK designed the study and drafted the manuscript. SD and LDK conducted the systematic review. FS evaluated pathological characteristics of PCNSL following CLIPPERS. MB provided evaluation of MRI findings. All authors analysed and interpreted data, critically revised, read and approved the final manuscript. CS and LDK oversaw this study.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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