Nationwide incidence and survival of retinoblastoma in the USA between 1996 and 2018: a review of 5730 cases

Retinoblastoma is the most common intraocular cancer in children, with significant variations in incidence and Survival. This study sought to evaluate nationwide retinoblastoma incidences and survival rates in the United States from 1996 to 2018. We extracted data from the North American Association of Central Cancer Registries (NACCR) and the National Program of Cancer Registries between 1996 and 2018. Cases were stratified by sex, age, race, Hispanic origin, urban or rural origin, stage of the disease, and state of residence and compared by laterality. We applied SEER*Stat version 8.4.0.1 software for age-adjusted incidence rates (AAIR) and annual average per cent change (AAPC) and JoinPoint version 4.9.1.0 software to assess incidence trends. Survival trends were evaluated with JPSurv online software. The study comprised 5730 patients with a crude incidence rate (CIR) of 0.89 per million. The incidence declined from 29.6 per million in the first year of life to 16.5 in the second year. The CIR for unilateral and bilateral diseases were 0.61 and 0.27, respectively. Males had a slightly higher and significant crude incidence of 0.93 versus 0.86 for females. Hispanics had the highest rate of 1.48 in general. Treatment data revealed chemotherapy usage in 39% of unilateral and 78% of bilateral patients. The 5-year cumulative relative Survival did not significantly differ between unilateral (96.8%) and bilateral (97.0%) patients. A slight but significant annual decline in CIR was observed, with a trend toward an increase in the percentage of patients diagnosed in the first year of life. This study indicated an overall decrease in incidence but a slight increase in early diagnosis. Findings underscore the need for consistent surveillance, early detection strategies, and personalized care to improve patient outcomes. The changes in the Survival in the unilateral disease require further investigations and mitigation strategies.

视网膜母细胞瘤（Retinoblastoma）是儿童最常见的眼内恶性肿瘤，其发病率与生存率存在显著差异。本研究旨在评估1996至2018年美国全国范围内视网膜母细胞瘤的发病率与生存率。我们从北美中央癌症登记协会（North American Association of Central Cancer Registries, NACCR）与国家癌症登记计划（National Program of Cancer Registries）中提取了1996至2018年的相关研究数据。研究人员按性别、年龄、种族、西班牙裔族裔、城乡居住地、疾病分期及居住州对病例进行分层，并依据肿瘤侧别开展组间对比分析。本研究使用SEER*Stat 8.4.0.1版本软件计算年龄调整发病率（age-adjusted incidence rates, AAIR）与年度平均变化百分比（annual average per cent change, AAPC），并采用JoinPoint 4.9.1.0版本软件分析发病率变化趋势。生存率变化趋势则通过JPSurv在线软件进行评估。本研究共纳入5730例患者，粗发病率（crude incidence rate, CIR）为每百万人0.89例。发病率从出生后第一年的每百万人29.6例降至出生后第二年的16.5例。单侧与双侧病变的粗发病率分别为0.61与0.27。男性粗发病率略高于女性，分别为0.93与0.86，差异具有统计学意义。西班牙裔族裔的总体粗发病率最高，达1.48。治疗数据显示，39%的单侧病变患者与78%的双侧病变患者接受了化疗。单侧（96.8%）与双侧（97.0%）患者的5年累积相对生存率无显著统计学差异。研究观察到粗发病率存在轻微但具有统计学意义的年度下降趋势，同时出生后第一年确诊的患者占比呈现上升趋势。本研究表明，视网膜母细胞瘤的总体发病率有所下降，但早期诊断率略有提升。研究结果强调，需持续开展肿瘤监测、优化早期筛查策略并实施个性化诊疗，以改善患者预后。单侧病变患者的生存率变化仍需进一步研究，并需制定相应的干预策略。