Background/aims To determine if patients with vitreoretinal lymphoma (VRL) and concomitant central nervous system lymphoma (CNSL) may present without brain MRI findings, but possess cerebrospinal fluid (CSF) suspicious for lymphoma. Methods This was a retrospective, single-centre, observational study evaluating patients with a diagnosis or suspicion of VRL seen at Memorial Sloan Kettering Cancer Center between 2006 and 2024. Patients were included if the final diagnosis was biopsy-proven CNSL and both MRI brain with and without contrast±CSF evaluation (obligatory for inclusion if MRI negative) were performed at the initial diagnostic workup. Patients were excluded if CNS disease treatment (brain, spine or CSF) preceded ocular disease. Patients with prior extra-CNS disease were included. Clinical records and radiographic imaging were retrospectively reviewed and relevant data were recorded for each patient. We evaluated the proportion of patients with MRI negative and CSF suspicious for lymphoma. Subgroup analysis included imaging features, pathology, treatment and disease course. Results We identified 65 patients. Of the 65 patients at the presentation of VRL, 30 had negative MRI brain and CSF, 16 had positive brain MRI and negative CSF and 8 had both positive MRI brain and CSF. 11 (16.9%) had CSF suspicious for lymphoma without positive findings on MRI of the brain. In this subgroup, the median age was 66 years (range 49–82) and 36% were female. 86% of these patients were asymptomatic neurologically. 73% underwent systemic treatment. At a mean 3 years follow-up, 91% of patients were living. Conclusion In patients with suspected VRL, it is possible to have CSF test positive for lymphoma in the context of negative brain MRI. This suggests, when evaluating VRL patients for concomitant CNS disease, CSF evaluation leads to earlier detection and systemic treatment, even when MRI brain findings are negative. In our cohort, an absence of CSF evaluation in the context of negative brain MRI could have missed 16.9% of patients with CNS lymphoma. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译：

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玻璃体视网膜淋巴瘤：初始诊断时脑脊髓液评估的重要性


背景/目标 确定玻璃体视网膜淋巴瘤 （VRL） 和伴随的中枢神经系统淋巴瘤 （CNSL） 患者是否可能没有脑部 MRI 结果，但具有疑似淋巴瘤的脑脊液 （CSF）。方法 这是一项回顾性、单中心、观察性研究，评估 2006 年至 2024 年间在纪念斯隆凯特琳癌症中心就诊的诊断或疑似 VRL 的患者。如果最终诊断是活检证实的 CNSL，并且在初始诊断性检查时进行了有和没有造影剂±CSF 评估的脑部 MRI 评估 （如果 MRI 阴性，则必须纳入） ，则纳入患者。如果 CNS 疾病治疗 （脑、脊柱或 CSF） 先于眼部疾病，则排除患者。包括既往患有 CNS 外疾病的患者。回顾性回顾性回顾临床记录和影像学检查，并记录每位患者的相关数据。我们评估了 MRI 阴性和 CSF 疑似淋巴瘤的患者比例。亚组分析包括影像学特征、病理学、治疗和病程。结果 我们确定了 65 例患者。在 VRL 就诊的 65 例患者中，30 例脑部 MRI 和 CSF 阴性，16 例脑部 MRI 阳性和 CSF 阴性，8 例脑部 MRI 和 CSF 均阳性。11 例 （16.9%） 怀疑 CSF 为淋巴瘤，但脑部 MRI 结果无阳性结果。在该亚组中，中位年龄为 66 岁 （范围 49-82），其中 36% 为女性。这些患者中 86% 在神经系统上无症状。73% 接受了全身治疗。在平均 3 年的随访中，91% 的患者存活。结论 在疑似 VRL 患者中，在脑部 MRI 阴性的情况下，脑脊液淋巴瘤检测呈阳性的可能性。 这表明，在评估 VRL 患者是否伴有 CNS 疾病时，即使脑部 MRI 结果为阴性，CSF 评估也可以更早地发现和全身治疗。在我们的队列中，在脑部 MRI 阴性的情况下缺乏 CSF 评估可能会漏掉 16.9% 的 CNS 淋巴瘤患者。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。