Primary vitreoretinal lymphoma: short review of the literature, results of a European survey and French guidelines of the LOC network for diagnosis, treatment and follow-up

Denis Malaise (1, 2) , Caroline Houillier (3) , Valérie Touitou (4) , Sylvain Choquet (4) , Karim Maloum (4) , Magali Le Garff-Tavernier (4) , Frédéric Davi (4) , Anne Vincent-Salomon (2) , Loïc Feuvret (4) , Khê Hoang-Xuan (3) , Nathalie Cassoux (2, 5) , Carole Soussain (6, 7)

1 LITO - Laboratoire d'Imagerie Translationnelle en Oncologie
2 Institut Curie [Paris]
3 ICM - Institut du Cerveau = Paris Brain Institute
4 CHU Pitié-Salpêtrière [AP-HP]
5 Biologie Cellulaire et Cancer
6 ICSC - Institut Curie - Saint Cloud
7 U932 - Immunité et cancer

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 highdose 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.

Mots clés: diagnosis and management challenges; European mapping; guidelines; primary vitreoretinal lymphoma; treatment

Domaines: Hématologie; Cancer; Médecine humaine et pathologie

Denis Malaise, Caroline Houillier, Valérie Touitou, Sylvain Choquet, Karim Maloum, et al.. Primary vitreoretinal lymphoma: short review of the literature, results of a European survey and French guidelines of the LOC network for diagnosis, treatment and follow-up. Current Opinion in Oncology, 2021, 33 (5), pp.420 - 431. ⟨10.1097/cco.0000000000000776⟩. ⟨hal-03559355⟩