Abstract
BACKGROUND: Vitreoretinal lymphoma (VRL) is regarded as a subtype of primary central nervous system lymphoma (PCNSL). Hence, extending progression-free survival (PFS) is crucial for enhancing the prognosis of VRL patients. Nevertheless, a lack of standard treatment options currently exists for VRL. This systematic review aims to explore the most optimal treatment strategy. METHODS: The methods for this systematic review and meta-analysis adhered to PRISMA guidelines and followed a protocol registered on the PROSPERO registry. A search was conducted on PubMed, Embase, and Scopus up to October 14, 2023, using predefined search terms. Primary endpoints included overall response rate (ORR) and complete response rate (CRR), while secondary endpoints comprised overall survival (OS) and progression-free survival (PFS). RESULTS: Thirty-seven studies comprising 801 patients were included in the meta-analysis. The pooled CRR was 85%, and the ORR was 93%. The pooled 1-year PFS was 83%, and the 2-year PFS was 58%. The 1-year OS was 92%, and the 2-year OS was 80%. The combined median PFS was 22.87 months, and the median OS was 51.19 months. Survival analysis of the extracted data showed significant associations between PFS and OS with systemic therapy (p = 0.00098 and p = 0.0091) and multi-strategy combination therapy (p = 0.0081 and p = 0.007); however, age, gender, and bilateral involvement exhibited no significant relationship (p > 0.05). CONCLUSIONS: In conclusion, while existing treatment strategies have led to higher remission rates and longer OS for VRL patients, PFS remains suboptimal. The primary focus of future clinical and basic research will be to explore effective treatment strategies for controlling disease recurrence or progression. TRIAL REGISTRATION: This meta-analysis was registered in the international prospective register of systematic reviews (PROSPERO) (CRD42023400305).