Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma

Denise Carbonell, Sarakshi Mahajan, Soon-Phaik Chee, Bianka Sobolewska, Rupesh Agrawal, Tanja Bülow, Vishali Gupta, Nicholas P. Jones, Massimo Accorinti, Mamta Agarwal, Tracy Batchelor, Jyotirmay Biswas, Luca Cimino, Ninette H tenDam-van Loon, Alejandra de-la-Torre, Shahar Frenkel, Jacob Pe’er, Michal Kramer, Elisabetta Miserocchi, Manabu MochizukiThomas Ness, James T. Rosenbaum, H. Nida Sen, Michael Simion, Helmut Sitter, Daniel V. Vasconcelos-Santos, Zohar Habot-Wilner, Sarah E. Coupland, José S. Pulido, Justine Smith, Jennifer E. Thorne, Manfred Zierhut, Study Group for Vitreoretinal Lymphoma Diagnostics

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22 Citations (Scopus)


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.
Original languageEnglish
Pages (from-to)507-520
Number of pages14
JournalOcular Immunology and Inflammation
Issue number3
Early online date3 Apr 2021
Publication statusPublished - 2021


  • Vitreoretinal lymphoma
  • Diagnosis
  • Consensus
  • Recommendations
  • consensus recommendations
  • diagnosis


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