Treatment Of Primary Central Nervous System Lymphom

Primary central nervous system lymphoma is a rare, extranodal form of non-Hodgkin lymphoma that is confined to the brain, spinal cord, eyes and leptomeninges.Survival rates have improved over the past few decades due to radiotherapy and chemotherapy regimens based on high-dose methotrexate. Improvement has also been made in treatment with addition of targeted drugs. Treatment has evolved from whole-brain radiotherapy as a single-modality treatment towards a high-dose methotrexate based, multi-agent chemotherapy regimens while WBRT is reserved for consolidation or given to relapsed patients as salvage treatment. Today, cure is a possibility for patients with PCNSL, but adverse neurocognitive sequelae caused by radiotherapy and disease relapse after treatment remain to be the major challenges. To address this,investigators have focused on improving drug therapy(for example:dose intensified chemotherapy consolidation approaches) in an attempt to reducing the dose of whole-brain radiotherapy or even omitting radiotherapy.Despite high chemosensitivity and radiosensitivity, remissions are frequently of short duration. About 35% to 60% of patients will relapse within two years after active treatment. In addition, 10% to 15% of patients have lymphoma refractory to first-line therapy. We need to select the appropriate salvage treatment for these patients. However, there is no exactly standard on decisions of which one is the best solution and the best dosage of induction, consolidation, salvage treatment programs.In fact, current therapeutic knowledge is only based on three randomized trials, some single-arm phase-II trials, a few meta-analyses, and some multicenter retrospective studies. Thus, available level of evidence is low, with lack of consensus on therapeutic decisions.In this manuscript, I discuss available clinical data on PCNSL in immunocompetent patients and provide recommendations for everyday practice and the rationale for future trials.