Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma

• Published in: Ocular immunology and inflammation Vol. 29; no. 3; pp. 507 - 520
• Main Authors: Carbonell, Denise, Mahajan, Sarakshi, Chee, Soon-Phaik, Sobolewska, Bianka, Agrawal, Rupesh, Bülow, Tanja, Gupta, Vishali, Jones, Nicholas P., Accorinti, Massimo, Agarwal, Mamta, Batchelor, Tracy, Biswas, Jyotirmay, Cimino, Luca, tenDam-van Loon, Ninette H., de-la-Torre, Alejandra, Frenkel, Shahar, Pe'er, Jacob, Kramer, Michal, Miserocchi, Elisabetta, Mochizuki, Manabu, Ness, Thomas, Rosenbaum, James T., Sen, H. Nida, Simion, Michael, Sitter, Helmut, Vasconcelos-Santos, Daniel V., Habot-Wilner, Zohar, Coupland, Sarah E., Pulido, José S., Smith, Justine, Thorne, Jennifer E., Zierhut, Manfred
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis 03.04.2021
• Subjects: Biomarkers, Tumor - metabolism; consensus recommendations; Delphi Technique; diagnosis; DNA Mutational Analysis; Humans; Interleukin-10 - metabolism; Interleukin-6 - metabolism; Intraocular Lymphoma - diagnosis; Intraocular Lymphoma - genetics; Intraocular Lymphoma - metabolism; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - genetics; Lymphoma, Large B-Cell, Diffuse - metabolism; Mutation, Missense; Myeloid Differentiation Factor 88 - genetics; Retinal Neoplasms - diagnosis; Retinal Neoplasms - genetics; Retinal Neoplasms - metabolism; Retrospective Studies; Surveys and Questionnaires; Vitreoretinal lymphoma; Vitreous Body - metabolism; Vitreous Body - pathology; Vitreoretinal lymphoma; consensus recommendations; diagnosis
• ISSN: 0927-3948; 1744-5078; 1744-5078
• DOI: 10.1080/09273948.2021.1878233

To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL). Literature was reviewed for reports supporting the diagnosis of VRL. A questionnaire (Delphi 1 round) was distributed to 28 participants. In the second round (Delphi 2), items of the questionnaire not reaching consensus (75% agreement) were discussed to finalize the recommendations. Presenting symptoms include floaters and painless loss of vision, vitreous cells organized into sheets or clumps. Retinal lesions are usually multifocal creamy/white in the outer retina. Other findings include retinal lesions with "leopard-skin" appearance and retinal pigment epithelium atrophy. Severe vitreous infiltration without macular edema is the most likely presentation. Diagnostic vitrectomy should be performed. Systemic corticosteroid should be discontinued at least 2 weeks before surgery. An interleukin (IL)-10:IL-6 ratio > 1, positive mutation for the myeloid differentiation primary response 88 gene and monoclonality are indicators of VRL. Multi-modal imaging (optical coherence tomography, fundus autofluorescence) are recommended. A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.