Current management of primary central nervous system lymphoma

Primary CNS lymphoma is rising in incidence in both the AIDS and non-AIDS populations. It is a non-Hodgkin's lymphoma that usually presents as a brain tumor, but the leptomeninges, eyes, and spinal cord also are frequently affected. Systemic lymphoma is not present, and comprehensive systemic staging is unnecessary, but appropriate neurologic staging is imperative. Standard therapy has been whole brain radiotherapy, giving a median survival of 12 to 18 months in non-AIDS patients, but only 2 to 5 months in AIDS patients. In non-AIDS patients, the addition of chemotherapy to radiotherapy has improved the prognosis, with median survivals of 30 to 45 months. Current protocols focus on the development of combination chemotherapy programs and reducing the dose of cranial radiotherapy to minimize late neurologic sequelae. The addition of chemotherapy to brain irradiation prolongs survival in some patients with AIDS-related disease, but median survival is not significantly improved.