Lymphomas of the Retina and Choroid

Lymphomas of the retina and choroid are rare but aggressive intraocular malignancies that most commonly represent a subset of primary central nervous system lymphoma (PCNSL) or, less frequently, systemic lymphoma with secondary ocular involvement. Retinal lymphoma, often referred to as primary vitreoretinal lymphoma, typically arises from diffuse large B-cell lineage and presents with chronic, relapsing vitritis that mimics uveitis, leading to frequent diagnostic delays. Choroidal lymphoma, in contrast, is usually a low-grade B-cell lymphoma and may occur as a primary ocular tumor or as part of systemic disease, often presenting with painless visual disturbances and characteristic choroidal infiltrates. Advances in multimodal imaging, including optical coherence tomography, fundus autofluorescence, fluorescein angiography, and ultrasonography, have improved clinical suspicion and localization of disease. Definitive diagnosis relies on cytologic, immunohistochemical, and molecular analysis of ocular specimens, such as vitreous or chorioretinal biopsy, though limited tissue yield and cellular fragility remain significant challenges. Emerging biomarkers, including interleukin profiles and genetic mutations, have shown promise in supporting diagnosis and monitoring disease activity. Management strategies depend on disease extent and include local therapies, such as intravitreal chemotherapy or ocular radiation, and systemic treatments for central nervous system or systemic involvement. Despite therapeutic advances, prognosis remains guarded, particularly for retinal lymphoma due to its strong association with CNS disease. Early recognition, multidisciplinary collaboration, and ongoing research into targeted therapies are essential to improving visual outcomes and overall survival in patients with retinal and choroidal lymphomas.