Intraocular lymphoma: report of three cases and review of the literature.

Three cases of intraocular lymphoma are presented. One patient had only ocular involvement, one had involvement of the eye and central nervous system, and in the third patient, ocular lymphoma developed 1 year after the diagnosis of a systemic lymphoma. One patient died before treatment could be initiated, but the other two patients responded well to local radiotherapy. Only one patient who received radiation to both eyes and the whole brain, followed by systemic chemotherapy, remains alive 4 years after diagnosis. Eighty-seven cases of intraocular lymphoma reported in the literature are reviewed. Only 16.7% of cases involved the eyes alone without central nervous system or systemic disease. In more than one-half of the cases (59.7%), the eye was the primary site of involvement. Craniotomy or enucleation was required for diagnosis in 52.7% of patients, and diagnosis frequently followed a significant period of delay during which time patients were treated unsuccessfully for uveitis or iritis. Death for most patients was due to progressive central nervous system involvement. Therefore, we recommend combined modality therapy with radiation to the whole brain and both eyes, followed by systemic chemotherapy with or without intrathecal medications.