Vitreoretinal Lymphoma: Changing Trends in Diagnosis and Local Treatment Modalities

Abstract

To report changing trends in treatment (external beam radiation therapy (EBRT) and intravitreal chemotherapy) from a single institution and report clinical and ocular imaging findings of patients (pts) with vitreoretinal lymphoma (VRL). A retrospective chart review of pts with non-responsive uveitis and vitritis was performed. Only pts with vitreous biopsy proven lymphoma were included. Data recorded included demographics, systemic lymphoma status and treatment, ocular symptoms and clinical findings, optical coherence tomography (OCT), fluorescein angiography (FA) and immunocytological findings, treatment methods intravitreal methotrexate 300 micrograms/0.05 ml, 1000 mcg of rituximab in 0.1 cc and EBRT) and response to treatment. Ocular and systemic lymphoma outcomes at last follow-up visit were also included. Twelve eyes of 8 pts were included in the study. All 8 pts were whites. The mean age of pts was 64.5 years (range 50-83). Central nervous system non-Hodgkin lymphoma (CNS-NHL) was present in 7 of 8 pts (87.5%). Four pts had bilateral disease at presentation (50%). Most common ocular symptoms were diminution of vision in 4 and floaters in 3 pts. Prior to presentation to our center, 50% pts were treated with topical steroids and other 50% did not receive any treatment. Iritis and uveitis were seen in 6 eyes (50%) and vitritis in 11 eyes (91.7%). Retinal infiltrates were present in 8 eyes (66.7%). Two eyes had choroidal lesions (16.7%), of which one was associated with exudative retinal detachment another with papillitis. On OCT, mean central foveal thickness was 270 (range 215-371) μ with presence of cystoid macular edema was present in 2 eyes, subretinal fluid in 1 and pigment epithelial irregularities in 3 eyes. Most common FA findings were macular edema in 6 eyes (60%), perivascular leakage in 2 (20%) and no leakage in 2 eyes (20%). Vitreous biopsy revealed B cell lymphoma in all patients (100%). Immunocytologic analysis in 2 pts showed elevated levels of IL-6 (26.7 pg/ml), IL-10 (12783.5 pg/ml) and IgH gene rearrangement suggestive of lymphoma. Three pts were treated with EBRT (36-45 Gy), of which one had primary VRL. Six eyes were treated with intravitreal methotrexate (range 2-15, mean 9.7). Two pts developed marked keratitis secondary to methotrexate toxicity (33.3%), one of which received intravitreal rituximab injections for persistent vitritis. None developed rituximab related side effects. The mean duration of follow-up was 37.8 months (range 4-96). VRL had resolved in 7 eyes (58.3%) and persistent in 5 eyes (41.7%). Systemic lymphoma was in remission in 5 pts (62.5%). One pt died due to advanced CNS NHL. Intravitreal chemotherapy provided good control rates for VRL pts in our limited series. OCT and FA are superior ocular imaging helpful tools in work-up. Pts with associated CNS NHL had poorer outcomes.