Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL). Methods: 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. Results: 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. Conclusions: A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma / Carbonell, D., Mahajan, S., Chee, S.-P., Sobolewska, B., Agrawal, R., Bulow, T., Gupta, V., Jones, N.P., Accorinti, M., Agarwal, M., Batchelor, T., Biswas, J., Cimino, L., tenDam-van Loon, N.H., de-la-Torre, A., Frenkel, S., Pe'Er, J., Kramer, M., Miserocchi, E., Mochizuki, M., et al.. - In: OCULAR IMMUNOLOGY AND INFLAMMATION. - ISSN 0927-3948. - 29:3(2021), pp. 507-520. [10.1080/09273948.2021.1878233]
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma
Cimino L.;
2021
Abstract
Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL). Methods: 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. Results: 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. Conclusions: A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.
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