Primary malignant lymphoma of the central nervous system--report of four long-term survivors.

Four of 47 patients treated between 1977 and 1993 for histologically confirmed primary malignant lymphoma of the central nervous system (non-Hodgkin's type of B-cell origin) achieved long-term survival for more than 5 years with a good quality of life. Three have remained disease-free for 9-12.5 years. The fourth achieved complete remission for more than 5 years before death from tumor recurrence. All four patients were treated with a standard therapeutic regimen, consisting of radiotherapy (50-60 Gy local and 30-40 Gy whole brain irradiation) followed by four to six courses of chemotherapy with cyclophosphamide, vincristine, adriamycin, and prednisolone at 4- to 8-week intervals. No further treatment was performed after remission had been obtained. No specific predictors for long-term survival including sex, age, tumor location, multiplicity of lesions, histology, or treatment modality was identified. All four patients showed an immediate tumor response to radiation. We recommend chemotherapy at increasing intervals as part of the post-therapeutic management of long-term, disease-free patients.