Clinical Features, Treatment, and Outcomes of Retinoblastoma in China

Abstract

Background This review determined clinical features and treatment outcomes of retinoblastoma patients in China, which ranks second in incidence globally and is among the countries listed to produce 4,000 new cases of the estimated world's retinoblastoma cases in 2023. Methods A search was done using different databases for literatures on retinoblastoma in China published from 2010 to 2020. The articles were then reviewed for clinical features, treatment, and outcomes. Results Ten articles that included 3,702 patients involving ∼4,412 eyes seen in China from 1957 to 2019 were analyzed. Median age at consult ranged from 18 to 30 months and mean lag of consultation was 4 to 6 months. More males were affected (58%). Seventy-nine percent had unilateral disease. Retinoblastoma was assumed intraocular in 4,123(89%) eyes with ≥996(22%) belonging to group E of International Intraocular Retinoblastoma Classification/International Classification of Retinoblastoma. Extraocular extension was present in 415 (9%) eyes with 845 patients having direct extraocular extension, while 54 had distant metastasis. Enucleation was the most used treatment procedure specially in unilateral disease done in at least 2,781 (74%) eyes. Median follow-up period ranged from 14 to 47 months. Functional vision was retained in 48 (2%) eyes. Globe salvage rate for group A to D eyes ranged from 56 to 100%. Highest globe salvage rate for group D was 87% and 70% for group E. Overall survival rate was 1,655/1898 (87%), ranging from 81 to 100%. Overall mortality was 4%. Discussion Clinical profile and management options for retinoblastoma in China changed overtime improving outcomes. Globe salvage and survival rate were high for those with intraocular disease.