LAUSR

INTRODUCTION Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (PCNSL) represents a relevant challenge in onco-hematology. Indeed, PCNSL has a specific molecular profile and peculiar biological characteristics that distinguish it from systemic DLBCL. Recently, several translational studies have allowed for significant improvement in the knowledge about the genomic and molecular profile of this rare entity. High-dose-methotrexate-based immune-chemotherapy followed by consolidation with whole-brain irradiation or autologous stem cell transplantation is the most commonly used therapeutic approach in PCNSL patients, but several efforts are still needed to improve the outcomes and management. AREAS COVERED This work provides an overview of the new biomarkers of PCNSL, focusing on their potential diagnostic, predictive and prognostic role. Publications in English language, peer-reviewed, high-quality international journals, were identified on PubMed. EXPERT OPINION Early diagnosis, a better antitumor response definition and recognition of new effective treatments are important research fields aiming to improve PCNSL outcome. The acquisition of new molecular and genomic knowledge in PCNSL has allowed for the attainment of promising diagnostic and prognostic tools as well as the development of clinical trials addressing new therapeutic approaches beyond chemotherapy agents, which have demonstrated activity in refractory/relapsed PCNSL and deserve to be investigated as part of first-line therapy.