Primary malignant lymphoma of the central nervous system

Between 1960 and 1983, 19 patients with primary malignant lymphoma of the central nervous system (CNS) were seen at McGill University Hospitals. The diagnosis was made at autopsy in 3 patients, and by biopsy in 16. Results of treatment were poor. All four patient who underwent surgery alone died within 2 months of diagnosis. Of 12 patients who underwent surgery and postoperative radiotherapy, 11 died between 2 and 56 months (median, 12 months) following diagnosis, and one is alive with disease at 47 months. Patterns of involvement at first recurrence and/or at autopsy were analyzed for 13 patients. Failure at the original site of involvement was unusual after treatment consisting of surgery and radiotherapy. In contrast, failure in the brain at sites other than those originally involved was common in spite of the use of whole brain irradiation. Local leptomeningeal involvement was seen in one patient whose diagnosis was made at autopsy, and cerebral spinal fluid seeding was seen in two additional patients, one within 1 month of diagnosis and one at relapse at 6 months after diagnosis. No patient developed disease outside the CNS. The limitations of current therapy for this disease are discussed, and certain suggestions made regarding the management of future patients with this diagnosis.