肝癌是全球疾病负担的一个重要因素，其中肝母细胞瘤是儿童最常见的肝脏肿瘤，90%的病例发生在生命的前5年内。尽管小儿肝癌很罕见，但对于小儿胃肠病学和肝病学的儿科医生和亚专科医生来说，了解小儿肝癌的流行病学和发病趋势非常重要。在本研究中，我们首先提供了 2000 年至 2021 年儿童期和青少年期肝母细胞瘤和肝癌的发病率和死亡率负担的估计值。肝癌负担及其可归因的危险因素是使用全球疾病负担研究的数据进行估计的（ GBD）2021。估计百分比变化显示2000年至2021年肝癌估计的趋势。年龄标准化率（ASR）和估计年度百分比变化（EAPC）用于测量肝母细胞瘤发病率和死亡率趋势。根据 GBD 框架，通过对 1000 次抽取的数据进行平均，得出所有估计的 95% 不确定性区间 (UI)，以及 95% UI 的下限和上限。全球范围内，从 2000 年到 2021 年，年龄为 5-19 岁肝癌发病例数和死亡例数从2449.2例（95%UI：2235.9-2689.8）下降到1692.9例（95%UI：1482.0-1992.5），2248.5例（95%UI：2053.7-2474.9）下降到1516.6例（95 ％UI：1322.1-1797.9），分别。与此同时，从2000年到2021年，20-24岁年龄组中，肝癌的发病例和死亡例分别从1453.5例（95%UI：1327.8-1609.4）下降到1285.1例（95%UI：1159.2-1447.2）和1432.3例。 （95% UI：1307.6-1585.7）至 1195.5（95% UI：1066.1-1355.2）。此外，5-19岁组肝癌患病率从41.9%（95% UI：18.7%-64.7%）下降到26.4%（95% UI：14.2%-39.1%），5-19岁组肝癌患病率从46.6%（ 20-24岁年龄段的95% UI：42.8%-51.5%）至36.5%（95% UI：33.1%-40.9%）。 2000年至2021年，在5-19岁年龄组中，乙型肝炎导致的肝癌发病比例从42.2%下降到37.9%，丙型肝炎导致的肝癌发病比例从1.1%上升到1.6%。此外，NASH诱发的肝癌发病比例从5.2%上升至9.4%，同期饮酒诱发的肝癌发病率也从0.5%上升至0.7%。从2000年到2021年，全球范围内肝母细胞瘤的发病病例和死亡病例从6131.8例（95% UI：5234.8-6961.9）下降到4045.6例（95% UI：3250-4995.8）和4059.2例（95% UI：3494.5-4621.2）分别为 2416（95% UI：1940.2-3022.5）。与年龄相关的性别特征存在一定差异，肝母细胞瘤发病数最高的是2岁至4岁的儿童，12个月至9岁的女性新发病例数较多。重要的是，肝母细胞瘤的发病率在1个月后开始急剧增加。本研究的结果对于儿童和青少年时期的肝脏健康政策和实践具有重要意义。为了减少肝癌的影响，有必要根据年龄和性别采取差异化的干预和推广策略。肝母细胞瘤的早期筛查和干预非常重要，特别是对于9岁以下人群。该研究得到了国家重点研发计划的支持

Liver cancer is a significant contributor to the global disease burden, of which hepatoblastomas are the most common liver tumors in children, with 90% of cases occurring within the first 5 years of life. It is important for pediatricians and subspecialists in pediatric gastroenterology and hepatology to have knowledge of the epidemiology and incidence trends of pediatric hepatic cancer, despite its rarity. In the present study, we first provide estimates of the incidence and mortality burden of hepatoblastoma and liver cancer from 2000 to 2021 in the childhood and adolescence.Liver cancer burden and its attributable risk factors were estimated using data from the Global Burden of Disease Study (GBD) 2021. Percentage change was estimated to show the trend of liver cancer estimates from 2000 to 2021. The age-standardized rate (ASR) and estimated annual percentage change (EAPC) were utilized for measuring hepatoblastomas incidence and deaths rate trends. In accordance with the GBD framework, 95% uncertainty intervals (UIs) for all estimates by averaging the data from 1000 draws, with the lower and upper bounds of the 95% UIs.Globally, from 2000 to 2021 in the age 5-19 years group, the incidence cases and deaths cases due to liver cancer decreased from 2449.2 (95% UI: 2235.9-2689.8) to 1692.9 (95% UI: 1482.0-1992.5) and 2248.5 (95% UI: 2053.7-2474.9) to 1516.6 (95% UI: 1322.1-1797.9), respectively. Meanwhile, from 2000 to 2021 in the age 20-24 years group, the incidence cases and deaths cases due to liver cancer decreased from 1453.5 (95% UI: 1327.8-1609.4) to 1285.1 (95% UI: 1159.2-1447.2) and 1432.3 (95% UI: 1307.6-1585.7) to 1195.5 (95% UI: 1066.1-1355.2), respectively. In addition, the prevalence of liver cancer decreased from 41.9% (95% UI: 18.7%-64.7%) to 26.4% (95% UI: 14.2%-39.1%) in the age 5-19 years group, and 46.6% (95% UI: 42.8%-51.5%) to 36.5% (95% UI: 33.1%-40.9%) in the age 20-24 years. From 2000 to 2021, in the age group of 5-19 years, the proportion of liver cancer incidence due to hepatitis B has decreased from 42.2% to 37.9%, while the proportion due to hepatitis C has increased from 1.1% to 1.6%. Additionally, there has been an increase in the proportion of NASH-induced liver cancer incidence from 5.2% to 9.4%, and alcohol use induced liver cancer incidence has also increased from 0.5% to 0.7% over the same period. Globally, from 2000 to 2021, the incidence cases and deaths cases due to hepatoblastoma decreased from 6131.8 (95% UI: 5234.8-6961.9) to 4045.6 (95% UI: 3250-4995.8) and 4059.2 (95% UI: 3494.5-4621.2) to 2416 (95% UI: 1940.2-3022.5), respectively. There was some variation in age-related sex-specific patterns, the highest number of hepatoblastoma incidence cases occurred in children between 2 and 4 years old and females in the age range of 12 months to 9 years had a higher number of new cases. Importantly, the incidence of hepatoblastoma was started to increase sharply after the age of 1 month.The results of the present study are significant for liver health policy and practice in childhood and adolescence. Differentiated intervention and outreach strategies based on age and gender would be necessary to reduce the impact of liver cancer. Early screening and interventions for hepatoblastoma is important especially in the population of under 9 years old.This study was supported by the National Key R&D Program of China (grant numbers 2023YFC2307000), National Natural Science Foundation of China [grant numbers 82170571 and 81974068], China Postdoctoral Science Foundation (grant numbers 2023M741283).© 2024 The Author(s).