Treatment Option Overview
In a prospective, randomized trial of 551 immunocompetent patients with newly diagnosed primary CNS lymphoma, all patients received induction chemotherapy with six cycles of high-dose methotrexate (4 g/m2) with or without ifosfamide. Upon completion of chemotherapy, responders were randomly assigned to WBRT (45 Gy) or to no treatment for complete response patients and cytarabine for partial response patients. There was no statistical difference in median OS with 32.4 months for patients receiving radiation therapy versus 37.1 months for those not receiving radiation (hazard ratio [HR] = 1.06; 95% confidence interval [CI], 0.80–1.40, P = .71).[Level of evidence: 1iiA] Treatment-related neurotoxicity was significantly worse on the radiation therapy arm, and such toxicity must be weighed against the possibility that the survival from chemotherapy alone may be marginally inferior to the survival when radiation is added.
Patients with acquired immunodeficiency syndrome (AIDS) associated primary CNS lymphoma usually have very advanced human immunodeficiency virus (HIV) infections with CD4 counts less than 50 cells/mm3. Consequently, most patients die of opportunistic infections regardless of therapy for the lymphoma. Groups that benefit most from radiation therapy, with or without antecedent chemotherapy, include those HIV-seropositive patients with no prior opportunistic infections or tumors for whom the CNS lymphoma is the AIDS-defining illness, and those patients with a good performance status, high CD4 lymphocyte count (>100mm3), and symptoms referable only to the CNS lymphoma.[32,41] Treatment of these patients requires special consideration. (Refer to the PDQ summary on AIDS-Related Lymphoma Treatment for more information.)
An international consortium performed a retrospective review of 83 HIV-negative patients with primary intraocular lymphoma. In selected patients with no evidence of disseminated CNS disease, localized therapy with intraocular methotrexate or ocular radiation therapy is associated with equivalent outcomes seen with systemic chemotherapy and/or WBRT. Localized therapy with intraocular methotrexate or ocular radiation therapy did not affect relapse rate, median PFS, or median OS as compared to systemic chemotherapy and/or WBRT.[Level of evidence: 3iiiDiv]
Current Clinical Trials