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Primary vitreoretinal lymphoma: prevalence, impact, and management challenges

Authors Venkatesh R, Bavaharan B, Mahendradas P, Yadav NK

Received 16 November 2018

Accepted for publication 23 January 2019

Published 14 February 2019 Volume 2019:13 Pages 353—364

DOI https://doi.org/10.2147/OPTH.S159014

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Andrew Yee

Peer reviewer comments 4

Editor who approved publication: Dr Scott Fraser


Ramesh Venkatesh,1 Bharathi Bavaharan,1 Padmamalini Mahendradas,2 Naresh Kumar Yadav1

1Department of Retina and Vitreous, Narayana Nethralaya, Bengaluru – 560010, India; 2Department of Uvea and Intraocular Inflammation, Narayana Nethralaya, Bengaluru – 560010, India

Abstract: Primary vitreoretinal lymphoma (PVRL) is a rare and potentially fatal intraocular malignancy. More than half of PVRL cases eventually involve the central nervous system (CNS). PVRL frequently masquerades as chronic uveitis. Advanced imaging tests, such as optical coherence tomography and fundus autofluorescence, have been applied in the diagnosis of PVRL. Histology and immunohistochemistry, in combination with molecular tests and IL-10 analysis, have been demonstrated as reliable in diagnosing PVRL. Mortality is high in patients with PVRL associated with CNS involvement, and relapses are common. The use of systemic chemotherapy in addition to the local therapies has proved to extend the mean survival time of these patients. Local therapies, including intravitreal injections of methotrexate and/or rituximab and low-dose radiotherapy to the eye, have been shown to be extremely effective in controlling intraocular lymphoma.

Keywords: CNS lymphoma, malignancy, ocular tumor, treatment, investigations, prognosis


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