Primary malignant lymphoma of the central nervous system. Results of treatment of 11 cases and review of the literature

Eleven patients with primary malignant lymphoma of the central nervous system (CNS) were treated at the Medical College of Wisconsin Affiliated Hospitals between 1964 and 1984. Three patients had a prior history of immunosuppressive therapy following renal transplantation. All patients had biopsy-proven disease and 10 of the 11 were treated with external radiation therapy. The doses to the primary tumor ranged from 34 to 59.4 Gray (Gy). Actuarial (life-table) survival rate was 82% at 1 year and 43% at 3 years. No recurrence was seen after 13 months. Eighty-six reports totaling 693 cases of primary malignant lymphoma of the CNS were found in the literature. Of these, 308 cases were treated with a combination of surgery and irradiation. Overall survival at 5 years for those patients who received more than 50 Gy compared with less than 50 Gy to the primary tumor was 42.3% versus 12.8% (p < 0.05). Twenty-one patients survived longer than 5 years. Late relapse was notable, with 10 (47.6%) of 21 tumors recurring between 5 and 12.5 years after diagnosis. Based on this review, a minimum of 50 Gy radiation to the primary tumor is recommended. While no statement regarding the efficacy of craniospinal irradiation or chemotherapy can be made in view of the small numbers, the use of craniospinal irradiation and/or systemic chemotherapy should be considered for future trials.