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Primary vitreoretinal lymphoma: recent advances and literature review

Primary vitreoretinal lymphoma is a rare B-cell intraocular malignancy, commonly considered as a subset of primary central nervous system (CNS) lymphoma. Brain dissemination occurs in 60–90% of patients and it is the cause of the high mortality and morbidity of the disease. PVRL involves the vitreous, the retina, and the retinal pigment epithelium (RPE). The majority of patients complain about hazy vision and floaters occurring for several months. PVRL commonly masquerades as posterior uveitis, with a misleading temporary response to steroids, responsible for its late diagnosis. Fundoscopy and advanced imaging tests such as optical coherence tomography (OCT), fluorescein angiography (FA) and fundus autofluorescence help in the diagnosis, as different peculiar findings have been described. Nevertheless, cytology and immunohistochemistry of vitreous samples are required to confirm PVRL diagnosis. Vitreous and aqueous interleukin analysis (IL-6/IL10 ratio) and mutated gene detection (MYD88 L265P) have recently demonstrated high reliability. Several treatments are available for PVRL, including methotrexate (MTX) and rituximab intravitreal (IV) injections, radiation therapy and systemic chemotherapy. However, due the rarity and the complexity of the disease, literature about vitreoretinal lymphoma is limited and definite guidelines are lacking. The aim of this review is to highlight crucial points and recent advances useful for the diagnosis, management and treatment. Indeed recently, novel imaging tools in conjunction with new molecular diagnostic techniques have helped in distinguish typical vitreoretinal lymphoma features. Furthermore, we present different experience on treatment and the available data on follow up. Herein, we try to address the lack of univocal consensus in management of patients with vitreoretinal lymphoma and the desperate need of multicenter studies to define the optimal management for PVRL, especially when CNS involvement has developed.