Key Words. Primary CNS lymphoma • Molecular genetics • Chemotherapy • Stem cell transplantation • Rituximab •

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors or independent peer reviewers. Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extranodal non-Hodgkin’s lymphomas (NHLs), primarily of B cell origin, whose in-cidence has markedly increased in the last three de-cades. Immunodeficiency is the main risk factor, but the large majority of patients are immunocompetent. Re-cent evidence suggests a specific tumorigenesis that may explain their particular clinical behavior compared with systemic NHL. The addition of i.v. high-dose meth-otrexate (MTX) chemotherapy to whole-brain radio-therapy (WBRT) has considerably improved th