@article{3106633, title = "Primary vitreoretinal lymphoma", author = "Kalogeropoulos, D. and Vartholomatos, G. and Mitra, A. and Elaraoud, I. and Ch'ng, S.W. and Zikou, A. and Papoudou-Bai, A. and Moschos, M.M. and Kanavaros, P. and Kalogeropoulos, C.", journal = "Saudi Journal of Ophthalmology", year = "2019", volume = "33", number = "1", pages = "66-80", publisher = "Elsevier B.V.", issn = "1319-4534", doi = "10.1016/j.sjopt.2018.12.008", keywords = "antineoplastic agent; cytokine; methotrexate; rituximab, animal model; aspiration; autofluorescence; B cell lymphoma; B scan; brain biopsy; cancer chemotherapy; cancer prognosis; cancer radiotherapy; cancer risk; carcinogenesis; cerebrospinal fluid examination; clinical examination; cytogenetics; cytopathology; differential diagnosis; flow cytometry; fluorescence angiography; histology; histopathology; human; immunohistochemistry; immunophenotyping; indocyanine green angiography; intraocular lymphoma; lymphoma cell; molecular diagnosis; molecular pathology; neuroimaging; nonhuman; ophthalmoscopy; optical coherence tomography; pars plana vitrectomy; primary tumor; primary vitreoretinal lymphoma; priority journal; recurrence risk; Review; uveitis; vitreoretinopathy; vitreous biopsy", abstract = "Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, which consists a subset of primary central system lymphoma (PCNSL) and the most common type of intraocular lymphoma. The involvement of eyes is estimated to be approximately 20% of PCNSL, but the brain involvement may be up to 80% of PVRL. Typically, PVRL is a high grade B-cell malignancy of the retina and needs to be assorted from choroidal low-grade B-cell lymphomas. Very often PVRL masquerades and can be erroneously diagnosed as chronic uveitis, white dot syndromes or other neoplasms. Establishing an accurate diagnosis may involve cytology/pathology, immunohistochemistry, flow cytometry, molecular pathology and cytokine profile analysis. There is inadequate information about PVRL's true incidence, ethnic/geographical variation and pathogenetic mechanisms. The therapeutic approach of PVRL involves aggressive chemotherapy and radiation therapy. Although PVRL tends to have a good response to the initial treatment, the prognosis is poor and the survival restricted due to the high relapse rates and CNS involvement. © 2019 The Authors" }