Abstract:

Primary vitreoretinal lymphoma (PVRL) is a rare and fatal ocular malignancy. PVRL is a subset of primary central nervous system lymphoma (PCNSL), and lack of characteristic clinical manifestations of the disease. PVRL requires invasive procedures for tissue diagnosis. Cytology/pathology, molecular pathology, immunohistochemistry, biophysical technology (flow cytometry), and cytokine analysis (interleukine-10) are often required. The gold standard for diagnosis is cellular immune pathology examination. Approaches to treatment have centered on systemic methotrexate-based chemotherapy. Rituximab is a new  meidicine in the treatment of PVRL, especially in the targeted therapy. Despite these advances, prognosis remain poor, and few patients remains disease-free >4.5 years after treatment.   (Int Rev Ophthalmol,  2015,  39:     422-427)