Abstract: Primary central nervous system lymphoma refers to a special type of nonHodgkin's lymphoma that is confined to the central nervous system without evidence of systemic spread. Its relatively low incidence and the limited pathological specimens  bring certain difficulties for further research. Genetic and molecular biology studies have confirmed its specific genetic background and molecular biological characteristics,  but the sample size of these studies is relatively small. Although the administration of highdose methotrexate has significantly improved the prognosis of the patients,  there is still no uniform optimal treatment, for most of the clinical studies are focused on phase Ⅱ singlecenter clinical trials and the sample size is relatively limited. Besides, there are no enough data to support clinical studies for treatment of the elderly and relapsed refractory patients. This article reviews the clinical and molecular biological characteristics and the treatment of primary central nervous system lymphoma.

Key words: lymphoma, central nervous system, cytogenetics, biological features, treatment