Primary vitreoretinal lymphoma an update on pathogenesis, diagnosis and treatmentReichstein, DavidCurrent Opinion in Ophthalmology: May 2016 - Volume 27 - Issue 3 - p 177–184 doi: 10.1097/ICU.0000000000000255 RETINAL, VITREOUS AND MACULAR DISORDERS: Edited by Brandon G. Busbee and John W. Kitchens Buy Abstract Author InformationAuthors Article MetricsMetrics Purpose of review 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. Recent findings 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. Summary 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. Tennessee Retina, 345 23rd Avenue North, Suite 350, Nashville, TN 37203, USA Correspondence to David Reichstein, Tennessee Retina, 345 23rd Avenue North, Suite 350, Nashville, TN 37203, USA. Tel: +1 615 983 6000; fax: +1 615 983 6010; e-mail: email@example.com Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.