PRIMARY VITREORETINAL LYMPHOMA: A REVIEW ON DIAGNOSIS AND MANAGEMENT CHALLENGES

Abstract

Primary vitreoretinal lymphoma is a rare type of primary central nervous system lymphoma and is most commonly a diffuse large B-cell lymphoma histologic subtype. It is exceedingly rare with approximately 400 new cases diagnosed annually and diagnosed between the 5th and 6th decades of life. Diagnosis is often delayed and mistaken for other etiologies and requires local tissue sampling orbital/neurological imaging by an ocular oncologist well versed in primary vitreoretinal lymphoma disease management. While local disease control is commonly achieved, disease relapse or dissemination to the central nervous system occurs in over 50% of patients. Although there is no agreed upon standard of care management, local therapy ranges from local injections of methotrexate and/or rituximab, low dose orbital radiation, and consideration of systemic chemotherapies if both eyes are involved, recurrent disease occurs, or central nervous system metastasis is found. Consolidation therapies including Bruton tyrosine kinase inhibitors, autologous hematopoietic stem cell transplantation, or radiation may be considered in recurrent local disease or with central nervous system dissemination. In this review, we focus on current diagnostic and management strategies, challenges, and future directions.