ABSTRACT
Primary CNS lymphoma (PCNSL) is a rare malignancy with peculiar clinical and biologic features,aggressive course,and unsatisfactory outcome.the particular microenvironment of this malignancy,and sanctuary sites where tumor cells grow undisturbed, strongly affects treatment efficacy. The execution of prospective trials is also difficult because of the rarity of the tumor and the impaired general condition and poor performance status of patients. Chemotherapy is indispensable for the treatment of PCNSL. High-dose methotrexate is the most effctive drug.PCNSL is sensitive to irradiation,but responses to radiotherapy alone are short-lived. MTX-based chemotherapy followed by whole-brain radiotherapy prolonged survival but is associated with delayed neurotoxicity especially in patients older than 60 years. A new approach is attempted to treat PCNSL with chemotherapy alone with defered radiotherapy in old patients with similar survival rencenfly. High-dose chemotherapy with autologous stem cell transplantation HDC/ASCT yielded promising results for recurent PCNSL.Clinical investigation with Temozolomide or rituximab have been reported.Further studies with these new drugs are needed.