Abstract
Background/aims: To report our five-year experience on vitreoretinal lymphoma (VRL) as a single-center tertiary hospital. Methods: The ophthalmic, cytopathology, and onco-hematologic records of patients with VRL consecutively seen from 2014 to 2019 were reviewed. Results: Fifty-nine eyes of 31 patients with large B-cell VRL were included. Eighty-one percent has developed central nervous system lymphoma at the end of follow-up. Several different imaging findings were noted, including vitritis, leopard spot appearance, Bruch's membrane/RPE infiltrations, and ellipsoid zone disruption. A variable combination of MYD88-L265P mutation in the aqueous and/or in the vitreous and positive cytology/histology allowed to reach a definite diagnosis in all the patients. Therapies included intravitreal injections of methotrexate and rituximab, systemic chemotherapy, pan-encephalic radiotherapy, and hematopoietic stem cell transplantation. Conclusion: No definite guidelines exist for VRL management. It is crucial to collect as much data as possible from tertiary referral hospitals, which suitably manage a conspicuous number of VRL patients.
Keywords:
CNS lymphoma; intravitreal chemotherapy; multimodal imaging; oncology; uveitis; vitreoretinal lymphoma.
MeSH terms
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Aged
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Aged, 80 and over
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Antimetabolites, Antineoplastic / therapeutic use
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Antineoplastic Agents, Immunological / therapeutic use
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Central Nervous System Neoplasms / diagnosis
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Central Nervous System Neoplasms / drug therapy
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Central Nervous System Neoplasms / genetics
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Central Nervous System Neoplasms / pathology*
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Female
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Humans
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Intraocular Lymphoma / diagnosis
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Intraocular Lymphoma / drug therapy
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Intraocular Lymphoma / genetics
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Intraocular Lymphoma / pathology*
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Intravitreal Injections
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Lymphoma, Large B-Cell, Diffuse / diagnosis
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Lymphoma, Large B-Cell, Diffuse / drug therapy
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Lymphoma, Large B-Cell, Diffuse / genetics
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Lymphoma, Large B-Cell, Diffuse / pathology*
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Lymphoma, Non-Hodgkin / diagnosis
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Lymphoma, Non-Hodgkin / drug therapy
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Lymphoma, Non-Hodgkin / genetics
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Lymphoma, Non-Hodgkin / pathology*
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Male
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Methotrexate / therapeutic use
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Middle Aged
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Mutation, Missense / genetics
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Myeloid Differentiation Factor 88 / genetics
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Retinal Neoplasms / diagnosis
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Retinal Neoplasms / drug therapy
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Retinal Neoplasms / genetics
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Retinal Neoplasms / pathology*
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Rituximab / therapeutic use
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Vitreous Body / pathology*
Substances
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Antimetabolites, Antineoplastic
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Antineoplastic Agents, Immunological
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MYD88 protein, human
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Myeloid Differentiation Factor 88
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Rituximab
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Methotrexate