Abstract

BackgroundPrimary central nervous system lymphoma (PCNSL) is a highly aggressive and rare extranodal non-Hodgkin lymphoma (NHL). The MSKCC and the IELSG scores represent the most widely used prognostic models, but many changes have occurred in therapeutic protocols since their development. Moreover, many PCNSL patients cannot be classified using the IELSG score. We thus aimed to create a novel, effective and feasible prognostic model for PCNSL.MethodsWe included 248 PCNSL patients diagnosed with PCNSL. Our primary endpoint was the overall survival (OS) and we used the receiver operating characteristic (ROC) analysis to determine the optimal prognostic cut-off value for LLR (lactate dehydrogenase-to-lymphocyte ratio), neutrophil-to-lymphocyte ratio (NLR) and derived neutrophil-to-lymphocyte ratio (dNLR). Variable associated with OS were evaluated by univariate and multivariate analyses. 124 out of 248 patients were randomly selected as the internal validation cohort.ResultsBy univariate analysis, an age >60 years, Eastern Cooperative Oncology Group performance status (ECOG PS) >1, treatment with radiotherapy alone, high-risk groups of Memorial Sloan Kettering Cancer Center (MSKCC) score, NLR >4.74, dNLR >3.29, and LLR >166.8 were significantly associated with a worse OS. By multivariate analysis, the MSKCC score and LLR were confirmed as independent prognostic parameters for poorer OS. OS, however, was not significantly different between low- and intermediate-risk groups according to the MSKCC score, while LLR proved to be prognostically relevant and was thus used to develop a novel, effective three-tier PCNSL scoring system. Of 124 patients, 84 patients with survival data and LLR data were successfully validated by newly established PCNSL LLR scoring system.ConclusionsIn the present study, we demonstrate that a high LLR represents an independent unfavorable prognostic parameter in PCNSL patients which can be integrated into an effective prognostic model.

Highlights

  • Primary central nervous system lymphoma (PCNSL) is defined as a pathologically confirmed primary lymphoma of the central nervous system, arising within the brain, leptomeninges, spinal cord and eyes, without systemic involvement; it represents a highly aggressive and rare extranodal non-Hodgkin lymphoma (NHL) with a poor prognosis

  • The International Extranodal Lymphoma Study Group (IELSG) found that an age >60 years, Eastern Cooperative Oncology Group performance status (ECOG PS) >1, high level of lactate dehydrogenase (LDH), elevated cerebrospinal fluid protein concentration and involvement of deep regions of the brain were significantly associated with a worse outcome

  • Univariate analysis showed that age, ECOG PS, treatment, Memorial Sloan Kettering Cancer Center (MSKCC) score, neutrophil-tolymphocyte ratio (NLR), derived neutrophil-tolymphocyte ratio (dNLR), and lactate dehydrogenase-to-lymphocyte ratio (LLR) were significantly associated with the overall survival (OS) of PCNSL patients

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Summary

Introduction

Primary central nervous system lymphoma (PCNSL) is defined as a pathologically confirmed primary lymphoma of the central nervous system, arising within the brain, leptomeninges, spinal cord and eyes, without systemic involvement; it represents a highly aggressive and rare extranodal non-Hodgkin lymphoma (NHL) with a poor prognosis. Two scoring systems are commonly used to predict the outcome of PCNSL patients: the International Extranodal Lymphoma Study Group (IELSG) score (6) and the Memorial Sloan Kettering Cancer Center (MSKCC) score (7). The IELSG found that an age >60 years, Eastern Cooperative Oncology Group performance status (ECOG PS) >1, high level of lactate dehydrogenase (LDH), elevated cerebrospinal fluid protein concentration and involvement of deep regions of the brain were significantly associated with a worse outcome. Age and Karnofsky performance score (KPS) were the only two variables included in the prognostic model, and they were used to stratify participants into low-, intermediate-, and high-risk groups (characterized by: age

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