Primary central nervous system lymphomas: a 30-year experience at a single institution

Primary central nervous system lymphoma (PCNSL) is a rare disease that has been increasing in frequency. Clinical, histologic, and immunohistochemical data from 64 cases of PCNSL seen at Duke University Medical Center since 1968 were reviewed and tumors were classified using the REAL classification system. Thirty-two patients were male and 32 were female, with a mean age of 57.1 years, ranging from 16 to 82 years. Large B-cell lymphoma represented overwhelming the majority of PCNSL, accounting for 81% of all cases. Phenotypic T-cell lymphomas were rare with only two cases over the course of the study. Epstein-Barr virus was detected only in the immunocompromised patients and was identified in 75% of those immunocompromised patients who were tested. Overall survival was poor with a mean survival of 357 days and median survival of 158 days. One- and three-year survival rates were 29.6% and 7.8%, respectively. Type of treatment, duration of symptoms, site of lesion, and histologic subtype were not significant prognostic indicators, whereas concurrent immunosuppression was the strongest predictor of poor outcome. In AIDS patients (which accounted for 21.9% of the study group), the median survival was 65 days, which was significantly different than that seen in the immunocompetent group of 217 days (P = .001).