Objectives: We report the clinical outcomes of 22 patients with primary intraocular lymphoma (PIOL) treated over a 15-year period.
Materials and methods: Patients with confirmed PIOL by central pathology review treated from 1994 to 2010 with isolated ocular (N=13) or central nervous system (CNS) plus ocular involvement (N=9) were included. Intraocular and CNS failure-free survival, relapse-free survival, and overall survival outcomes were analyzed.
Results: Median follow-up was 29.0 (range, 10.2 to 96.4) months. Sixteen patients (9 with isolated ocular, 7 with ocular and CNS disease) received combined modality therapy (CMT) consisting of systemic chemotherapy (usually high-dose methotrexate based) and orbital +/- whole-brain radiation. Two patients were treated with chemotherapy and 4 with local ocular therapy alone. Among patients with isolated ocular versus CNS involvement, CNS failure-free survival was 79% versus 57%, and intraocular failures were 62% versus 78% at 24 months. Median relapse-free survival was 34.0 versus 21.3 months (P=0.368), and overall survival 43.4 versus 30.3 months (P=0.744), respectively. Three patients treated with CMT (2 with isolated ocular and 1 with CNS involvement) with >1-year follow-up alive at the time of analysis never relapsed, and one remains disease-free >4.5 years after treatment.
Conclusions: In this series of patients with PIOL+/- CNS disease, CNS and intraocular relapse were common. A trend toward better survival was seen among patients with isolated ocular presentation, and a limited number of long-term disease-free survivors seen after CMT.