恶性骨肿瘤是一组罕见的恶性肿瘤，我们的研究旨在根据监测、流行病学和结果数据（Surveillance, Epidemiology and End Results database）更新最近的流行病学估计。纳入了2000年至2019年诊断为恶性骨肿瘤的患者，并对其特征进行了回顾性描述。计算了有限持续期患病率、年龄调整后发病率和死亡率，并分析了年百分比变化以量化其变化速率。最后，描绘了观测生存率和相对生存率。还进行了肿瘤类型、年龄、性别、肿瘤分级、原发肿瘤部位和分期的亚组分析。结果方面，选取了11655名符合条件的恶性骨肿瘤患者。骨肉瘤是最常见的肿瘤类型，其次是软骨肉瘤、尤文恶性肉瘤和脊索瘤。从2000年（0.00069％）到2018年（0.00749％），恶性骨肿瘤的有限持续期患病率有所增加。在研究期间，所有患者的年龄调整发病率保持稳定，而骨肉瘤的发病率最高。亚组中死亡率有所不同，而老年患者（超过64岁）的死亡率最高，与其他年龄组相比。在所有骨肿瘤中，10年观察生存率和相对生存率分别为58.0％和61.9％。软骨肉瘤患者的生存结果最好，其次是骨肉瘤、尤文恶性肉瘤、脊索瘤和其他骨肿瘤。总之，不同肿瘤类型以及其他人口统计学和临床病理学变量的发病率和死亡率表现出不同的流行病学特征，这为进一步调整医疗资源提供了潜在建议。©2023 UICC.

Malignant bone tumors are a group of rare malignant tumors and our study aimed to update the recent epidemiologic estimates based on the Surveillance, Epidemiology and End Results database. Patients diagnosed with malignant bone tumors from 2000 to 2019 were included and their characteristics were retrospectively described. The limited-duration prevalence, annual age-adjusted incidence and mortality were calculated, and the annual percentage changes were analyzed to quantify the rate change. Finally, observed survival and relative survival rate were illustrated. Subgroup analysis across tumor type, age, gender, tumor Grade, primary tumor site and stage was also performed. As for results, a total of 11 655 eligible patients with malignant bone tumor were selected. Osteosarcoma was the most common tumor type, followed by chondrosarcoma, Ewing sarcoma and chordoma. The estimated limited-duration prevalence of malignant bone tumors increased from 2000 (0.00069%) to 2018 (0.00749%). Steady age-adjusted incidence was observed in all patients during the study period while the highest rate occurred in osteosarcoma. Mortality rates differed in subgroups while elder patients (older than 64 years) presented the highest mortality rate compared to other age groups. In all bone tumors, the 10-year observed survival and relative survival rates were 58.0% and 61.9%, respectively. Chondrosarcoma patients had the best survival outcome, followed by osteosarcoma, Ewing sarcoma, chordoma and other bone tumors. In conclusion, different epidemiologic performance in incidence and mortality was observed across tumor type as well as other demographic and clinicopathological variables, which provide potential suggestion for further adjustment of medical resource.© 2023 UICC.