Primary central nervous system lymphoma

Primary CNS lymphomas (PCNSL) represent a subgroup of malignancies with specific characteristics, aggressive course, and unsatisfactory outcome in contrast with other lymphomas comparable for tumour burden and/or histological type. Despite a high chemo- and radiosensitivity, remissions are frequently shortlasting, mainly because the blood brain-barrier limits the access of many drugs to the CNS. Moreover, survivor patients are at high risk of developing severe treatment-related toxicity, mainly disabling neurotoxicity, raising the question of how to balance therapy intensification with side-effects control. Although the prognosis remains poor, it has significantly improved over the past two decades as a result of better treatment strategies with a curative aim. Surgery has no impact on survival, and is reserved to diagnosis by stereotactic biopsy. Actual front-line therapy consists of high-dose methotrexate-based poly-chemotherapy. The optimal drugs combination has not yet been identified even if there is a suggestion for a synergistic role for the adjunction of cytarabine, thiotepa, and rituximab. Radiotherapy retains an important role as salvage therapy in refractory/relapsing patients, while its use is more debated in the setting of response consolidation in patients who achieve a complete remission after induction chemotherapy. High-dose chemotherapy supported by autologous stem-cell transplantation is increasingly used as an effective method aimed to control microscopic disease, and the pros and contras of this approach are outlined. (C) 2017 Elsevier B.V. All rights reserved.