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

Area of Science:

• Ophthalmology
• Hematology
• Oncology

Background:

• Vitreoretinal lymphoma (VRL) is a rare extranodal non-Hodgkin lymphoma.
• Accurate diagnosis and timely management are crucial for patient outcomes.

Purpose of the Study:

• To review current evidence on clinical features, imaging, diagnostics, and management of VRL.
• To provide updated information on treatment options for VRL.

Main Methods:

• Literature review of available treatment options for VRL.
• Analysis of diagnostic techniques including vitreous biopsy, cytology, immunohistochemistry, flow cytometry, cytokine profiling, gene rearrangement analysis, and MYD88 mutation detection.
• Evaluation of multimodal imaging (e.g., OCT) in diagnosis and monitoring.

Main Results:

• Diagnosis requires vitreous biopsy with ancillary molecular and cytologic tests.
• Multimodal imaging aids in suspicion, biopsy guidance, and treatment monitoring.
• Management includes intravitreal chemotherapy, radiation, and systemic chemotherapy; relapse and CNS involvement are common, affecting prognosis.
• High-dose methotrexate-based chemotherapy is favored for VRL with CNS disease.
• Emerging diagnostics (metagenomic sequencing) and therapies (CAR-T) show promise.

Conclusions:

• VRL management necessitates a multidisciplinary approach integrating diagnostics, imaging, and tailored therapy.
• Standardized diagnostic and management protocols are needed, requiring prospective multicenter studies.