Background Retinoblastoma is the most common intraocular malignancy in childhood. Despite one-third of cases occurring in Africa, little is known of the outcomes on the continent. This study aims to explore survival and globe salvage outcomes and identify their risk factors across a large cohort of patients from the African continent. Methods A 3-year prospective, observational study was conducted. Kaplan-Meier survival analysis was used to investigate the risk of globe loss and death from retinoblastoma in Africa. Cox regression was used to identify risk factors associated with these outcomes. Results A total of 958 patients from 41 African countries and 66 participating centres were enrolled in the study. The survival rate was 78.2% at 1 year and 66.2% at 3 years after diagnosis. Cox regression showed a higher risk of death with the most advanced clinical stage (cT4, HR=6.29 vs cT2, p<0.001). The risk of losing at least one eye after diagnosis was 50% within 4 months and 72.6% within 3 years. Higher risk of enucleation was associated with a higher clinical stage compared with cT1 (cT3, HR=4.11, p=0.001; cT4, HR=3.77, p=0.005). Conclusion Nearly one in every four children diagnosed with retinoblastoma in African participating centres succumb to retinoblastoma within 1 year. There is also high morbidity associated with the diagnosis as a large majority of patients require eye removal surgery. The outcome of disease in children with retinoblastoma in Africa is poor compared with other continents and requires prompt intervention by increasing efforts to improve survival and eye salvage outcomes. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译：

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非洲大陆 41 个国家的视网膜母细胞瘤生存和眼球摘除结果


背景 视网膜母细胞瘤是儿童期最常见的眼内恶性肿瘤。尽管三分之一的病例发生在非洲，但人们对非洲大陆的结果知之甚少。本研究旨在探索生存和全球挽救结果，并确定来自非洲大陆的大量患者队列的风险因素。方法 进行了一项为期 3 年的前瞻性观察研究。Kaplan-Meier 生存分析用于调查非洲视网膜母细胞瘤导致全球丢失和死亡的风险。采用 Cox 回归确定与这些结局相关的危险因素。结果 共有来自 41 个非洲国家和 66 个参与中心的 958 例患者被纳入研究。诊断后 1 年生存率为 78.2%，3 年生存率为 66.2%。Cox 回归显示临床晚期死亡风险较高 （cT4，HR=6.29 vs cT2，p<0.001）。诊断后至少失去一只眼睛的风险在 4 个月内为 50%，在 3 年内为 72.6%。与 cT1 相比，较高的剜除风险与较高的临床分期相关 （cT3，HR=4.11，p=0.001;cT4，HR=3.77，p=0.005）。结论 在非洲参与中心，近四分之一被诊断患有视网膜母细胞瘤的儿童在 1 年内死于视网膜母细胞瘤。与诊断相关的发病率也很高，因为绝大多数患者需要摘除眼睛手术。与其他大陆相比，非洲视网膜母细胞瘤患儿的疾病预后较差，需要通过加大努力提高生存率和挽眼结果来及时干预。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。