Primary Vitreoretinal Lymphoma: Management of Isolated Ocular Disease.

BACKGROUND The prognosis for patients with primary vitreoretinal is dismal. The close association of primary vitreoretinal lymphoma with primary central nervous system lymphoma is responsible for high rates of mortality. Traditional treatments consist of systemic chemotherapy and whole-brain radiotherapy. The optimal approach for the treatment of isolated primary vitreoretinal lymphoma is unclear. METHODS A review of the relevant medical and scientific literature was performed, focusing on the clinical features of primary vitreoretinal lymphoma and the progress made in the management of isolated ocular disease. RESULTS Ocular treatment options for primary vitreoretinal lymphoma have recently expanded with the addition of intravitreal chemotherapeutic agents and localized radiation. Based on several retrospective reports, a general shift has been made toward local therapy (eg, orbital radiotherapy, intravitreal chemotherapy) for ocular disease. No prospective, randomized clinical trials yet exist to guide therapy. CONCLUSIONS Optimal treatment regimens for isolated primary vitreoretinal lymphoma continue to evolve. Further investigations into novel therapies and protocols are needed to decrease recurrence rates, reduce or prevent central nervous system involvement, and improve rates of overall survival.