Treatment of Intraocular Lymphoma

Retrospective reviews of selected patients with primary intraocular lymphoma and no evidence of disseminated central nervous system (CNS) disease showed that localized therapy with intraocular methotrexate or ocular radiation therapy or systemic therapy with rituximab were effective in clearing lymphoma cells from the eye. However, most patients had subsequent CNS relapse.[1,2][Level of evidence C3] Anecdotal series reported lower relapse rates when high-dose methotrexate was added, but prospective multicenter trials with even retrospective controls do not exist.[1,2][Level of evidence D]

Relapsing disease in the rest of the CNS is treated with the same options listed for primary CNS lymphoma in the brain. In a phase III randomized study of whole-brain radiation therapy, patients with intraocular disease and concomitant brain involvement had a worse prognosis than those with brain involvement alone (19 patients with both, 391 patients with brain involvement only).[3]