Jiang Tingting 1 , Li Ruiwen 2 , Liu Shixue 1 , Gu Junxiang 1 , Chen Wenwen 1 , Zhang Ting 1 , Huang Xin 1 , Xu Gezhi 1 , Chang Qing 1
  • 1. Department of Ophthalmology, Eye-ENT Hospital, Fudan University, Shanghai 200031, China;
  • 2. Department of Nursing, Eye-ENT Hospital, Fudan University, Shanghai 200031, China;
  • Jiang Tingting and Li Ruiwen are contributed equally to the article;
Chang Qing, Email: qngchang@aliyun.com
Lymphoma; Eye neoplasms; Central nervous system neoplasms; Chemoradiotherapy; Prognosis
10.3760/cma.j.cn511434-20220310-00126
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Objective 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.

Citation： Jiang Tingting, Li Ruiwen, Liu Shixue, Gu Junxiang, Chen Wenwen, Zhang Ting, Huang Xin, Xu Gezhi, Chang Qing. The diagnosis and treatment of primary vitreoretinal lymphoma: 10 years of experience. Chinese Journal of Ocular Fundus Diseases, 2022, 38(5): 376-381. doi: 10.3760/cma.j.cn511434-20220310-00126 Copy

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