Uveal lymphoma: Clinical features, diagnostic studies, treatment selection, and outcomes

Objective To describe the clinical features, ancillary diagnostic studies, and treatment selection in a cohort of patients with uveal lymphoma. Design Retrospective clinical review. Participants A total of 22 patients (34 affected eyes) diagnosed with uveal lymphoma between 1997 and 2013. Methods Data were collected regarding patient characteristics, clinical features on ophthalmic examination, ancillary imaging studies, and primary treatment selection. Main Outcome Measures Relapse defined as lymphoma recurrence in the initial site of presentation, the contralateral eye, or other systemic site and overall survival. Results Fifteen patients were male (68.2%). Median age at diagnosis was 68.0 years. The choroid was involved in 21 cases (95.5%), and 1 case (4.5%) was ciliochoroidal. Other ocular adnexal structures were affected in 13 patients (59.1%), including the conjunctiva in 4 (18.2%), the orbit in 7 (31.8%), and both the conjunctiva and orbit in 2 (9.1%). Bilateral disease was present in 12 patients (54.5%). The most common presenting symptom was decreased vision in 15 patients (68.2%). The median delay in diagnosis was 4.0 months. Yellow-white choroidal infiltrates were observed on fundus examination in 34 eyes (100.0%) with corresponding hypofluorescence in 100% of cases when indocyanine green angiography was performed. Infiltrates were located anterior to the arcades (67.6%), most commonly in a diffuse (32.4%) or superotemporal (32.4%) distribution. B-scan ultrasonography detected extrascleral extension in 22 patients (75.9%) with a pattern of crescentic thickening in 19 (86.4%). Extranodal marginal zone lymphoma was the predominant (76.2%) histologic subtype. External beam radiotherapy (72.7%) was most commonly chosen for primary treatment. Systemic imaging at the time of diagnosis revealed that the majority of cases (77.3%) were localized to the eye; none of the patients developed new systemic disease (median follow-up, 30.3 months). Conclusions Uveal lymphoma has distinctive clinical features. Overlap with ocular adnexal structures is common, and ancillary imaging is essential for evaluating the full extent of ocular disease and presence of systemic involvement.