Primary CNS lymphoma. Progress in the diagnostics and therapy

Background. Some important knowledge has recently been gained on primary central nervous system lymphomas (PCNSL) despite its rarity. Goal. This article summarizes the most relevant progress in the diagnostics and therapy of PCNSL and discusses future directions. Material and methods. Reference articles in the English language literature were studied with respect to future approaches in PCNSL. Results. New diagnostic methods in cerebrospinal fluid have been developed to facilitate lymphoma diagnosis; however, their value still has to be validated. A better immunohistological and molecular characterization of PCNSL will probably result in identification of new therapeutic targets. The only phase III trial for PCNSL completed so far did not demonstrate a survival advantage with whole brain irradiation after high-dose methotrexate (HDMTX)-based chemotherapy as compared to chemotherapy alone. The optimal primary chemotherapy has not yet been established due to a lack of results from randomized trials. Non-comparative studies suggest a superiority of combined polychemotherapy over HDMTX monotherapy. Future therapeutic developments are directed towards consolidation of HDMTX-based induction chemotherapy with noncross-resistant conventional chemotherapy or high-dose chemotherapy with autologous stem cell transplantation. An important goal of all therapies for PCNSL is to avoid delayed neurotoxicity. Discussion. Further improvement of diagnostics and well-designed comparative studies, including new drugs when possible are still needed to define the optimal management of this still frequently prognostically unfavorable disease.