Primary vitreoretinal lymphoma: an update on pathogenesis, diagnosis and treatment

Primary vitreoretinal lymphoma (PVRL) is a subset of primary central nervous system lymphoma in which disease primarily affects the uvea, retina, vitreous and optic nerve. This review discusses recent efforts to clarify the disease's pathogenesis, its diagnosis and its optimal treatment.

PVRL typically masquerades as a chronic intermediate uveitis in older individuals. Unambiguous diagnosis requires cytologic demonstration of malignant cells in a vitreous or chorioretinal specimen. However, cytokine analysis demonstrating increased interleukin 10 (IL 10) levels or increased IL-10:IL-6 ratio in the aqueous or vitreous, flow cytometry demonstrating a monoclonal cell population, molecular analysis demonstrating gene rearrangements or translocations or combinations of several techniques can be used effectively to aid in diagnosis. Treatment is aimed at eradication of disease within the eyes and prevention of central nervous system (CNS) lymphoma. Whether this should be done with local therapy alone (globe irradiation or intravitreal chemotherapy such as methotrexate or rituximab), or with systemic chemotherapy remains a source of debate. Even with high-dose systemic chemotherapy, CNS disease is prone to recurrence and has a poor prognosis.

New techniques and innovative treatment strategies may streamline time to definitive diagnosis and may lead to prolonged survival with better vision in patients with PVRL.