Vitreoretinal Lymphoma

Simple Summary Vitreoretinal lymphoma is a variant of primary CNS lymphoma involving the retina and/or the vitreous. At the time of presentation, CNS involvement occurs in up to one-third of patients. However, 50-90% of patients develop a CNS and/or spinal cord disease within one year. Therefore, it is important to frequently examine and recognize the early symptoms of CNS involvement. This review summarizes the clinical signs, ocular diagnosis and treatment of vitreoretinal lymphoma. Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL.

Automated summary

Vitreoretinal lymphoma is a rare variant of primary central nervous system lymphoma with up to 50% of patients developing CNS lymphoma within a year. Diagnosis is challenging due to nonspecific symptoms and there is no established treatment protocol.