Abstract: Background Retinoblastoma(Rb) is a common malignant tumor in childhood. In developing countries where 90% of children with Rb are located, reducing deaths is still a challenge, and the quality of life of children has received little attention.Objective To summarize the efficacy, safety and quality of life of children with unilateral intraocular retinoblastoma, and provide evidence for optimizing the treatment plan and improving the safety and effectiveness of treatment.DesignRetrospective non-randomized controlled study.Methods To retrospectively analyze the clinical data of children with unilateral intraocular retinoblastoma admitted to Beijing Children's Hospital, Capital Medical University from November 6, 2009 to September 5, 2019. The last follow-up date was April 20, 2020. The chemotherapy program was the RB-2009 program of Beijing Children's Hospital, which was improved according to the Canadian multi-center RB 2003 program, and 810 nm and 532 nm laser, condenser and other technologies were used for local treatment. The eye-preservation rate, survival rate, cause of death, toxic and side effects, etc. were analyzed, and the Child Quality of Life Universal Scale and the Child Quality of Life Cancer Module Scale were used to conduct quality of life surveys for children older than 5 years of age.Main outcome measures Five year overall survival(OS) rate and life quality.Results A total of 6,262 cases of Rb were included and 168 patients were followed up for 8 to 125 months (median time 51 months). There were 1 case in stage B, 3 cases in stage C, 95 cases in stage D, and 69 cases in stage E and the total course of chemotherapy was 1 to 12 cycles(mean 4.2± 2.2 courses).Enucleation was performed in 89 (53.0%) with 42 cases in stage D and 47 in stage E. Among them, 30 cases (33.7%) were directly enucleated, and 59 cases (66.3%) were enucleated after chemotherapy. Seventy-nine cases (47.0%) were preserved with 53(55.79%) in stage D, and 22 (31.9%) in stage E. The 5-year expected OS of all children was 95.2%. The expected 5-year OS was 95.5% in children with eye enucleation and 94.9% in children with preservation treatment. The differences were not statistically significant(P=0.78). Five patients (3.0%) suffered from reversible grade Ⅰ hearing impairment, and no grade Ⅱ-Ⅳ hearing impairment occurred. All children developed bone marrow suppression, and 6 patients received platelet transfusion (3.6%), and no infection-related death occurred. Among 106 patients completing the quality of life survey, the score of children with enucleation was lower than that of children with eye preservation in questions about self-perception, society and role. The differences were statistically significant(P＜0.05).There were 8 deaths with 5 cases in stage D and 4 cases in stage E, 2 of which died after giving up treatment due to economic reasons, and 6 of which died of intracranial metastasis after relapse.Conclusion VEC (Vincristine, Etoposide and Carboplatin) regimen combined with systemic chemotherapy and local treatment is safe and effective for children with unilateral intraocular retinoblastoma. Intracranial metastasis remains the most common cause of Rb-related death. The results of a survey on the quality of life of children over 5 years of age show that the eye enucleation children have a reduced quality of life in terms of appearance, self-feeling, society and role.

Key words: Retinoblastoma, Survival rate, Quality of life