摘要目的:探讨十年间复旦大学附属眼耳鼻喉科医院诊治的原发性玻璃体视网膜淋巴瘤（PVRL）患者的临床特征、治疗及预后情况。方法:回顾性临床研究。2011年至2021年于复旦大学附属眼耳鼻喉科医院检查确诊并接受治疗的PVRL患者67例126只眼纳入研究。其中，男性23例（34.3%，23/67 ），女性44例（65.7%，44/67 ）；平均年龄57.1岁。双眼59例（88.1%，59/67），单眼8例（11.9%，8/67）。眼部初诊时，有明确原发性中枢神经系统淋巴瘤（PCNSL）病史22例；头颅影像学检查发现颅内病灶5例；无中枢神经系统受累40例。因误诊葡萄膜炎给予糖皮质激素治疗20例。所有患者均接受玻璃体腔注射甲氨蝶呤（IVM）治疗，治疗方案为诱导期每周2次，持续2周；巩固期每周1次，持续1个月；维持期每个月1次。伴PCNSL或双眼患者同时接受全身化学药物治疗（化疗），部分联合脑部放射治疗（放疗）。随访时间平均39.3个月。回顾分析患者临床表现、治疗及预后情况。治疗前后视力比较采用 t检验。 结果:眼部初诊时已有明确PCNSL病史的22例，颅内诊断至眼部确诊时间平均为22.9个月；最初无中枢神经系统受累的40例中，有14例在随访过程中出现中枢神经系统受累（20.9%，14/67 ），眼部确诊至颅内诊断的时间平均为9.9个月。126只眼中，伴眼前节炎症42只眼（33.3%，42/126）。玻璃体炎型、视网膜型、玻璃体视网膜型分别为58 （46.0%，58/126）、7 （5.6%，7/126）、61 （48.4%，61/126）只眼，其中同时伴视神经受累9只眼（7.1%，9/126 ）。接受IVM治疗平均12次。IVM联合全身化疗59例（88.1%，59/67 ），其中16例同时联合脑部放疗。所有患者完成治疗周期后，病情均完全缓解（100.0%，67/67 ）。治疗后，眼部复发21只眼（16.7%，21/126）；颅内复发22例（32.8%，22/67 ）；死亡8例（11.9%，8/67）。患者无进展生存期平均为23.7个月；生存时间平均为43.6个月；5年总体生存率为72.5%。结论:PVRL表现复杂多样，多伴有中枢神经系统受累；其可分为玻璃体炎型、视网膜型和玻璃体视网膜型，视神经可同时受累；IVM联合全身治疗，病情可完全缓解。

abstractsObjective:To investigate the clinical characteristics, treatment and prognosis of primary vitreoretinal lymphoma (PVRL) diagnosed and treated in our hospital during the past 10 years.Methods:A retrospective clinical study. From 2011 to 2021, 126 eyes of 67 patients with PVRL who were diagnosed and treated in Department of Ophthalmology, Eye-ENT Hospital, Fudan University were included in the study. Among them, there were 23 males (34.3%, 23/67) and 44 females (65.7%, 44/67); the average age was 57.1 years. There were 59 cases with both eyes (88.1%, 59/67) and 8 cases with one eye (11.9%, 8/67). At the initial eye diagnosis, 22 cases had a clear history of primary central nervous system lymphoma (PCNSL); 5 cases were found to have intracranial lesions by head imaging examination; 40 cases had no central nervous system involvement. Twenty cases were treated with glucocorticoids due to misdiagnosed uveitis. All patients received intravitreal injection of methotrexate (IVM) treatment. The treatment regimen was twice a week in the induction period for 2 weeks, once a week in the consolidation period for 1 month, and once a month in the maintenance period. Patients with PCNSL or both eyes received concurrent systemic chemotherapy (chemotherapy), and some in combination with radiation therapy to the brain (radiotherapy). The mean follow-up time was 39.3 months. The clinical manifestations, treatment and prognosis of the patients were retrospectively analyzed. The visual acuity before and after treatment was compared by t test. Results:Among the 22 cases with a clear history of PCNSL at the initial eye diagnosis, the average time from intracranial diagnosis to eye diagnosis was 22.9 months. Among the 40 cases without central nervous system involvement at first, 14 cases (20.9%, 14/67) developed central nervous system lesions during follow-up period. The mean time from ocular diagnosis to intracranial diagnosis was 9.9 months. Among the 126 eyes, 42 eyes (33.3%, 42/126) had anterior segment inflammation. vitreous inflammation type, retinal type, and vitreous retinal type were 58 (46.0%, 58/126), 7 (5.6%, 7/126), and 61 (48.4%, 61/126) eyes, and 9 of them (7.1%, 9/126) had optic nerve involvement at the same time. Patients received an average of 12 IVM treatments. IVM combined with systemic chemotherapy in 59 cases (88.1%, 59/67), of which 16 cases were combined with brain radiotherapy. All patients achieved complete remission after completing the treatment cycle (100.0%, 67/67). After treatment, 21 eyes (16.7%, 21/126) had ocular recurrence; 22 (32.8%, 22/67) had intracranial recurrence; 8 cases (11.9%, 8/67) died. The mean progression-free survival of patients was 23.7 months; the mean survival time was 43.6 months; the 5-year overall survival rate was 72.5%.Conclusions:The manifestations of PVRL are complex and diverse, and most of them are accompanied by involvement of the central nervous system. It can be divided into vitreitis type, retinal type and vitreoretinal type, and the optic nerve can be involved at the same time; IVM combined with systemic treatment can completely relieve the disease.