LYMPHOMA: Edited by Dominique BronPrimary vitreoretinal lymphoma: short review of the literature, results of a European survey and French guidelines of the LOC network for diagnosis, treatment and follow-upMalaise, Denisa,b,c; Houillier, Carolined,e; Touitou, Valériee,f; Choquet, Sylvaine,g; Maloum, Karime,h; Le Garff-Tavernier, Magalie,h; Davi, Frédérice,h; Vincent-Salomon, Annei; Feuvret, Loïce,j; Hoang-Xuan, Khêd,e; Cassoux, Nathaliea,c,e,k; Soussain, Carolec,e,l,m Author Information aInstitut Curie, Ophthalmology Department, Paris bInstitut Curie, PSL University, Inserm U1288, Laboratoire d’Imagerie Translationnelle en Oncologie, Orsay cMember of European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet) dSorbonne Université, IHU, ICM, Department of Neuro-Oncology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris eFrench Network for Oculo-cerebral Lymphomas (LOC network) fSorbonne Université, Department of Ophthalmology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris gSorbonne Université, Department of Clinical Hematology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris hSorbonne Université, Department of Biological Hematology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris iInstitut Curie, PSL Research University, Department of Pathology, Department of Theranostic and Diagnostic Medicine jSorbonne Université, Department of Radiotherapy, Hôpital Pitié- Salpêtrière, Assistance Publique-Hôpitaux de Paris kUniversité de Paris, UMR 144 CNRS lInstitut Curie, Site de Saint-Cloud, Hematology Department, Saint-Cloud mINSERM U932 Institut Curie, PSL Research University, Paris, France Correspondence to Carole Soussain, MD, PhD, Institut Curie, Hématologie, 35 rue Dailly 92210 Saint-Cloud, France. Tel: +331 47 11 1536; e-mail: [email protected] Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.co-oncology.com). Current Opinion in Oncology: September 2021 - Volume 33 - Issue 5 - p 420-431 doi: 10.1097/CCO.0000000000000776 Buy SDC Metrics Abstract Purpose of review The aim of this study was to highlight the diagnostic and management challenges of primary vitreoretinal lymphoma (PVRL) through a review of the literature and a European survey on real-life practices for PVRL. Recent findings The care of PVRL patients is heterogeneous between specialists and countries. Upfront systemic treatment based on high-dose methotrexate chemotherapy, with or without local treatment, might reduce or delay the risk of brain relapse. Ibrutinib, lenalidomide with or without rituximab, and temozolomide are effective for patients with relapsed/refractory PVRL and should be tested as first-line treatments. Summary The prognosis of PVRL remains dismal. No firm conclusion regarding optimal treatment can yet be drawn. The risk of brain relapse remains high. Diagnostic procedures and assessment of therapeutic responses need to be homogenized. Collaboration between specialists involved in PVRL and multicentric prospective therapeutic studies are strongly needed. The recommendations of the French group for primary oculocerebral lymphoma (LOC network) are provided, as a basis for further European collaborative work. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.