Primary intraocular lymphoma: Clinical features, diagnosis and treatment

Primary intraocular lymphoma is a high-grade malignant lymphoid neoplasm, a subtype of central nervous system (CNS) lymphomas. It is mostly a diffuse large B-cell lymphoma. Approximately 19% of cases debut simultaneously with CNS involvement. There are two variants: uveal lymphoma and vitreoretinal lymphoma, with the latter being the most frequent and aggressive. Its diagnosis presents a challenge, as it behaves like uveitis, which delays the diagnostic suspicion. Due to limitations in vitreous cytological study, a series of complementar y tests exist to support the diagnosis: immunohistochemistry, flow cytometry, measurement of interleukins IL-6 and IL-10, among others. Several treatment alternatives have been established, but the focus is on intravitreal chemotherapy, which can be combined with systemic chemotherapy or radiotherapy. Despite advances in the diagnostic process, the prognosis remains poor. This review aims to characterize primary ocular lymphoma to enhance our understanding of this condition and enable early diagnostic suspicion.