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Review

Vitreoretinal Lymphoma

1
Center of Ophthalmology, University of Tuebingen, 72076 Tuebingen, Germany
2
Singapore National Eye Centre, 11 Third Hospital Avenue, Singapore 168751, Singapore
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Singapore Eye Research Institute, 11 Third Hospital Avenue, Singapore 168751, Singapore
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Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 168751, Singapore
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Duke-NUS Medical School, Singapore 168751, Singapore
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Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
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College of Medicine & Public Health, Flinders University, Adelaide 5042, Australia
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Institute for Pathology and Neuropathology, University of Tuebingen, 72076 Tuebingen, Germany
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Miyata Eye Hospital, Miyakonojo, Miyazaki 885-0051, Japan
*
Author to whom correspondence should be addressed.
Academic Editors: Nathalie Cassoux and Ryuya Yamanaka
Cancers 2021, 13(16), 3921; https://doi.org/10.3390/cancers13163921
Received: 10 June 2021 / Revised: 16 July 2021 / Accepted: 20 July 2021 / Published: 4 August 2021
(This article belongs to the Special Issue Primary CNS Lymphomas: Diagnosis and Treatment)
Vitreoretinal lymphoma is a variant of primary CNS lymphoma involving the retina and/or the vitreous. At the time of presentation, CNS involvement occurs in up to one-third of patients. However, 50–90% of patients develop a CNS and/or spinal cord disease within one year. Therefore, it is important to frequently examine and recognize the early symptoms of CNS involvement. This review summarizes the clinical signs, ocular diagnosis and treatment of vitreoretinal lymphoma.
Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL. View Full-Text
Keywords: vitreoretinal lymphoma; CNS lymphoma; IL-10/IL-6 ratio; MYD88; methotrexate vitreoretinal lymphoma; CNS lymphoma; IL-10/IL-6 ratio; MYD88; methotrexate
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MDPI and ACS Style

Sobolewska, B.; Chee, S.-P.; Zaguia, F.; Goldstein, D.A.; Smith, J.R.; Fend, F.; Mochizuki, M.; Zierhut, M. Vitreoretinal Lymphoma. Cancers 2021, 13, 3921. https://doi.org/10.3390/cancers13163921

AMA Style

Sobolewska B, Chee S-P, Zaguia F, Goldstein DA, Smith JR, Fend F, Mochizuki M, Zierhut M. Vitreoretinal Lymphoma. Cancers. 2021; 13(16):3921. https://doi.org/10.3390/cancers13163921

Chicago/Turabian Style

Sobolewska, Bianka, Soon-Phaik Chee, Fatma Zaguia, Debra Anne Goldstein, Justine R. Smith, Falko Fend, Manabu Mochizuki, and Manfred Zierhut. 2021. "Vitreoretinal Lymphoma" Cancers 13, no. 16: 3921. https://doi.org/10.3390/cancers13163921

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