本研究估计了安大略省接受治疗的脊索瘤患者的发病率、治疗模式和总体生存率。进行了一项为期 17 年（2003-2019 年）的基于人群的队列研究，包括安大略省癌症登记处的所有经组织学证实患有脊索瘤的患者。主要关注的结果是年龄标准化年发病率、总生存率以及放射治疗、化疗和开放切除率。在研究期间，共有 208 名患者被诊断为脊索瘤：97 名患者患有颅底脊索瘤，37 名患者患有活动性脊索瘤。脊柱脊索瘤 65 例，骶骨盆脊索瘤 65 例。共有 133 名患者接受了开放手术或内窥镜手术治疗，其中 99 名患者还接受了某种形式的放射治疗。在 17 年的研究期间，年平均年龄标准化发病率为每 1000 万例 12.04 例（95% CI 为每 1000 万例 9.31-14.78 例）。研究期间年发病率没有显着变化（平均年百分比变化 2.27，95% CI -1.74 至 6.44；p = 0.25）。在研究期间，接受放射治疗或化疗的几率每年显着增加 8%（95% CI 每年 1%-16%，p = 0.036）。接受开放切除的几率每年显着下降 14%（95% CI 每年 8%-20%，p < 0.001）。颅底脊索瘤患者接受内窥镜手术的几率每年增加 38%（95% CI 每年 22%-60%，p < 0.001），而在研究中，仅接受活检的患者的几率没有显着变化周期（p = 0.684）。脊索瘤诊断后，5年、10年和15年总生存概率分别为0.74（95% CI 0.69-0.81）、0.58（95% CI 0.51-0.67）和0.48（95% CI 0.40至0.59） ， 分别。死亡风险与诊断年份（p = 0.126）或解剖位置（p = 0.712，颅底与活动脊柱脊索瘤；p = 0.518 颅底与骶骨盆脊索瘤）之间没有显着相关性。脊索瘤是一种罕见疾病，没有2003 年至 2019 年间，年平均发病率发生了显着变化。在此期间，微创治疗方式有所增加，特别是颅底脊索瘤。许多患者的总生存期超过 10 年，在整个研究期间死亡风险没有变化。

This study estimates the incidence, treatment patterns, and overall survival for patients with chordoma treated in Ontario.A 17-year (2003-2019) population-based cohort study was conducted, including all patients in the Ontario Cancer Registry with histologically proven chordoma. Primary outcomes of interest were age-standardized annual incidence, overall survival, and rates of radiation therapy, chemotherapy, and open resection.A total of 208 patients were diagnosed with chordoma over the study period: 97 patients with skull base chordoma, 37 with mobile spine chordoma, and 65 with sacropelvic chordoma. A total of 133 patients were treated with either open or endoscopic surgery, of whom 99 were also treated with some form of radiation therapy. Across the 17-year study period, the average annual age-standardized incidence was 12.04 cases per 10 million (95% CI 9.31-14.78 cases per 10 million). There was no significant change in the annual incidence rate over the study period (average annual percent change 2.27, 95% CI -1.74 to 6.44; p = 0.25). The odds of receiving radiation therapy or chemotherapy significantly increased by 8% per year (95% CI 1%-16% per year, p = 0.036) over the study period. The odds of receiving open resection significantly decreased by 14% per year (95% CI 8%-20% per year, p < 0.001). The odds of receiving endoscopic surgery among patients with skull base chordoma increased by 38% per year (95% CI 22%-60% per year, p < 0.001), while the odds of patients receiving biopsy alone did not change significantly over the study period (p = 0.684). After diagnosis of chordoma, the 5-, 10-, and 15-year overall survival probabilities were 0.74 (95% CI 0.69-0.81), 0.58 (95% CI 0.51-0.67), and 0.48 (95% CI 0.40 to 0.59), respectively. There was no significant association between hazard of death and year of diagnosis (p = 0.126) or anatomical location (p = 0.712, skull base vs mobile spine chordoma; p = 0.518 skull base vs sacropelvic chordoma).Chordoma is a rare disease with no significant change in the average annual incidence rate between 2003 to 2019. During this time, treatment with less invasive modalities increased, particularly for skull base chordoma. Overall survival exceeds 10 years for many patients, with no change in the hazard of death across the study period.