To investigate the clinical features and prognosis of primary intraocular lymphoma (PIOL).

A retrospective review of medical records was performed in 9 patients who were diagnosed and treated as PIOL in the Department of Ophthalmology, Seoul National University Hospital.

Among patients who were enrolled in the study, 14 eyes were examined. Thirteen eyes (92.9%) showed yellowish subretinal or choroidal infiltrates which is a characteristic finding of PIOL in fundus examination and fluorescein angiography. Three patients presented with ocular symptoms initially, and 5 patients later presented with central nerve system (CNS) involvement. Only 1 patient showed PIOL without CNS involvement. Among 6 patients (9 eyes) that received systemic chemotherapy or ocular irradiation, 5 patients (7 eyes, 77.8%) responded. Among those patients, 3 patients (4 eyes) showed relapse of PIOL. Five patients died during the mean follow-up period of 43.3 months, and the median survival time was 47 months.

The most common characteristic fundus finding of PIOL is subretinal or choroidal infiltration. Ocular irradiation combined with systemic chemotherapy is the first method of treatment, although long-term prognosis is poor.