Institut Curie, Ophthalmology Department, Paris.
Institut Curie, PSL University, Inserm U1288, Laboratoire d'Imagerie Translationnelle en Oncologie, Orsay.
Curr Opin Oncol. 2021 Sep 1;33(5):420-431. doi: 10.1097/CCO.0000000000000776.
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.
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.
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.
本研究旨在通过文献复习和欧洲真实世界原发性眼内淋巴瘤（PVRL）实践调查，强调 PVRL 的诊断和治疗挑战。
PVRL 患者的治疗在专家和国家之间存在差异。基于大剂量甲氨蝶呤化疗的初始系统性治疗，联合或不联合局部治疗，可能会降低或延迟脑复发的风险。伊布替尼、来那度胺联合或不联合利妥昔单抗、替莫唑胺对复发性/难治性 PVRL 患者有效，应作为一线治疗进行检测。
PVRL 的预后仍然很差。目前还不能确定最佳治疗方法。脑复发的风险仍然很高。诊断程序和治疗反应的评估需要标准化。需要在参与 PVRL 的专家之间进行合作，并开展多中心前瞻性治疗研究。本文提供了法国原发性眼脑淋巴瘤小组（LOC 网络）的建议，作为进一步开展欧洲合作的基础。
