Primary central nervous system (CNS) lymphoma is an unusual but increasingly frequent brain tumor being identified by neurologists. Considerable improvements in survival have been accomplished by the addition of chemotherapy to cranial radiotherapy. In addition, many patients achieve substantial disease free survival with chemotherapy alone, and survival is superior to radiotherapy alone. Currently, every patient should be considered for chemotherapy as the first line of treatment. Subsequent cranial radiotherapy may or may not be necessary depending upon the patient's clinical condition, age and response to initial chemotherapy. Intensification of multi-agent chemotherapy is under study and it is anticipated that the incidence of neurotoxicity will reduce with elimination of combined modality treatment.
