Primary vitreoretinal lymphoma (PVRL) is a variant of primary central nervous system lymphoma (PCNSL), mostly a high-grade, extra-nodal, non-Hodgkin’s lymphoma. Diffuse large B-cell lymphoma (DLBCL) represents the predominant subtype, while rare cases of T-cell and natural killer (NK) cell origin can occur. The hallmark clinical features include vitreous cells and subretinal pigment epithelial (RPE) infiltrates of lymphoma. PVRL frequently masquerades as uveitis, therefore establishing the diagnosis can be challenging. Vitreous or subretinal biopsy remains the gold standard for diagnosis. The majority of patients with PVRL ultimately develop PCNSL which is an aggressive malignancy associated with poor overall survival.
KeywordsOptical Coherence Tomography Retinal Pigment Epithelium Cerebral Spinal Fluid Primary Central Nervous System Lymphoma Cystoid Macular Edema
- Chan CC, Rubenstein JL, Coupland SE, Davis JL, Harbour JW, Johnston PB, Cassoux N, Touitou V, Smith JR, Batchelor TT, Pulido JS. Primary vitreoretinal lymphoma: a report from an international primary central nervous system lymphoma collaborative group symposium. Oncologist. 2011;16(11):1589–99.CrossRefPubMedPubMedCentralGoogle Scholar