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Review
. 2021 Sep 1;33(5):420-431.
doi: 10.1097/CCO.0000000000000776.

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   3 , Caroline Houillier  4   5 , Valérie Touitou  5   6 , Sylvain Choquet  5   7 , Karim Maloum  5   8 , Magali Le Garff-Tavernier  5   8 , Frédéric Davi  5   8 , Anne Vincent-Salomon  9 , Loïc Feuvret  5   10 , Khê Hoang-Xuan  4   5 , Nathalie Cassoux  1   3   5   11 , Carole Soussain  3   5   12   13 ; LOC network and the ERN-EuroBloodNet
Affiliations
Free article
Review

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 et al. Curr Opin Oncol. .
Free article

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.

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References

    1. Coupland SE, Damato B. Understanding intraocular lymphomas. Clin Exp Ophthalmol 2008; 36:564–578.
    1. Chan C, Rubenstein JL, Coupland SE, et al. Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group Symposium. Oncologist 2011; 16:1589–1599.
    1. Lam M, Touitou V, Choquet S, et al. Intravenous high-dose methotrexate based systemic therapy in the treatment of isolated primary vitreoretinal lymphoma: an LOC network study. Am J Hematol 2021; 96:823–833.
    1. Grimm SA, Pulido JS, Jahnke K, et al. Primary intraocular lymphoma: an international primary central nervous system lymphoma collaborative group report. Ann Oncol 2007; 18:1851–1855.
    1. Touitou V, Daussy C, Bodaghi B, et al. Impaired Th1/Tc1 cytokine production of tumor-infiltrating lymphocytes in a model of primary intraocular B-cell lymphoma. Investig Ophthalmol Vis Sci 2007; 48:3223–3229.