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.