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In 1983, an association between Epstein-Barr viral infection and CNS lymphoma cells was established and led to speculation that the Epstein-Barr virus may play a role in the induction of CNS lymphoma (42,43). A growing body of evidence supports this contention. In a comparison of cerebrospinal fluid from patients with AIDS-related primary CNS lymphoma and those with non-AIDS-related primary CNS lymphoma, the polymerase chain reaction for Epstein-Barr virus genomic sequences was far more common in the group with AIDS (100% sensitive, 98.5% specific) (44). Further investigation with a mouse model revealed that Epstein-Barr virus-induced proteins are more likely to be found in AIDS-related primary CNS lymphoma than in non-AIDS-related forms.
Infection with other viruses, such as the cytomegalovirus, may also play a role in the development of CNS lymphoma in immunocompromised patients. Morgello et al (45) described a series of AIDS patients with primary CNS lymphoma in which CNS infections were found in 63% of the cases and 60% of these infections were caused by cytomegalovirus. In contrast, no CNS infections were seen in patients with primary CNS lymphoma who did not have AIDS (45). This intriguing prospect of an infectious cause may help explain the difficulty that early pathologists (who often believed that the histopathologic characteristics of these tumors were related to infection) encountered when classifying CNS lymphoma and continues to require further study.
There are no reported familial cases of primary CNS lymphoma in immunocompetent patients, in contrast to glioblastoma multiforme, which is a high-grade cerebral neoplasm that is associated with inherited genetic alteration in Li-Fraumeni syndrome (9,46).
Pathogenesis