Vitreoretinal Lymphoma: A Comprehensive Clinical Review and Current Standards in Management

Aditya Maitray; Pukhraj Rishi; Christopher D Conrady; Elaine Binkley; Basil K. Williams Jr; Steven Yeh; Maura Di Nicola; Paul T Finger

doi:10.1177/24741264261474159

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Vitreoretinal Lymphoma: A Comprehensive Clinical Review and Current Standards in Management

Journal article

Open access

Peer reviewed

Vitreoretinal Lymphoma: A Comprehensive Clinical Review and Current Standards in Management

Aditya Maitray, Pukhraj Rishi, Christopher D Conrady, Elaine Binkley, Basil K. Williams Jr, Steven Yeh, Maura Di Nicola and Paul T Finger

Journal of vitreoretinal diseases (Print)

08/13/2026

DOI: 10.1177/24741264261474159

PMID: 42602195

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Abstract

To summarize current evidence on clinical features, multimodal imaging findings, diagnostic techniques, and management strategies for vitreoretinal lymphoma. A literature review was performed to provide updated information on available treatment options for vitreoretinal lymphoma. Diagnosis of vitreoretinal lymphoma requires vitreous biopsy, with or without retinal/subretinal tissue, for cytology and immunohistochemistry, along with ancillary tests such as flow cytometry, cytokine profiling (interleukin-10/interleukin-6 ratio >1), immunoglobulin heavy chain gene rearrangement analysis, and detection of the MYD88 L265P mutation. Optical coherence tomography and other multimodal imaging techniques have become increasingly useful in raising suspicion, guiding biopsy, and monitoring treatment response. No standardized treatment protocol exists for isolated vitreoretinal lymphoma. Management options include intravitreal chemotherapy (methotrexate and/or rituximab), radiation therapy, and systemic chemotherapy, often showing a good initial response, but relapse and subsequent central nervous system (CNS) involvement are common, resulting in poor overall prognosis and survival. For vitreoretinal lymphoma with CNS disease, current strategies favor high-dose methotrexate-based systemic chemotherapy, with or without intrathecal chemotherapy; whole-brain radiation is generally reserved as rescue therapy. Emerging directions for earlier diagnosis include metagenomic deep sequencing, and chimeric antigen receptor T-cell (CAR-T) therapy has shown promise for treatment of selected relapsed/refractory cases of primary CNS lymphoma with a potential to prolong survival. Treatment of vitreoretinal lymphoma requires a multidisciplinary, individualized approach that integrates multimodal imaging, cytologic and molecular diagnostics, CNS evaluation, and tailored local or systemic therapy. Prospective multicenter studies are needed to refine diagnostic algorithms and standardize management.

Chemotherapy

eye

malignancy

radiation

vitreoretinal lymphoma

lymphoma

methotrexate

central nervous system lymphoma

rituximab

Details

Title: Subtitle: Vitreoretinal Lymphoma: A Comprehensive Clinical Review and Current Standards in Management
Creators: Aditya Maitray - Aravind Eye Hospital
Pukhraj Rishi - University of Nebraska Medical Center
Christopher D Conrady - University of Nebraska Medical Center
Elaine Binkley - University of Iowa
Basil K. Williams Jr - University of Miami Health System
Steven Yeh - University of Nebraska Medical Center
Maura Di Nicola - University of Miami
Paul T Finger - Eye Cancer Foundation
Resource Type: Journal article
Publication Details: Journal of vitreoretinal diseases (Print)
DOI: 10.1177/24741264261474159
PMID: 42602195
NLM abbreviation: J Vitreoretin Dis
ISSN: 2474-1272
eISSN: 2474-1272
Publisher: Sage
Language: English
Electronic publication date: 08/13/2026
Academic Unit: Ophthalmology and Visual Sciences
Record Identifier: 9985218373002771

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