Chordoma: incidence and survival patterns in the United States, 1973-1995
Chordoma: incidence and survival patterns in the United States, 1973-1995
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DOI：
10.1023/a:1008947301735
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发表时间：
2001-01-01
影响因子：
2.3
通讯作者：
Parry, DM
中科院分区：
文献类型：
--
作者：
McMaster, ML;Goldstein, AM;Parry, DM
背景：脊索瘤是一种由脊索残余引起的罕见肿瘤，迄今为止仅通过单一机构的病例系列或小型人群调查进行了描述。方法：我们使用1973-1995年美国国家癌症研究所监测、流行病学和最终结果（SEER）项目的数据，计算400例显微镜下确诊脊索瘤的年龄调整发病率和生存率，并得出病例分布和第二癌风险的信息。结果：年龄校正脊索瘤发病率（IR）为0.08 / 10万，与年龄有关，男性（IR 0.10）比女性（IR 0.06）更常见，在< 40岁和黑人患者中罕见。在中轴骨骼中，32%的病例位于颅骨，32.8%位于脊柱，29.2%位于骶骨。年轻（< 26岁；p = 0.0001）和女性（p = 0.037）与颅内表现的可能性较大相关。脊索瘤后第二原发癌症的总体风险没有增加。中位生存期为6.29年；5年和10年相对生存率分别为67.6%和39.9%。与其他骨肉瘤的比较揭示了两种发育性肿瘤脊索瘤和尤文氏肉瘤发病率的种族差异。结论：本研究为美国脊索瘤的发病率和生存模式提供了新的数据。需要更多的流行病学研究来阐明这种罕见的、独特的肿瘤的遗传和环境决定因素。
Background: Chordoma, a rare tumor arising from notochordal remnants, has been described to date only by single-institution case series or small population-based surveys.Methods: We used data from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute, 1973-1995, to calculate age-adjusted incidence and survival rates for 400 cases of microscopically confirmed chordoma and to derive information regarding case distribution and risk of second cancer.Results: The age-adjusted chordoma incidence rate (IR) of 0.08 per 100,000 was age-dependent, more common in males (IR 0.10) than females (IR 0.06) and rare among patients aged < 40 years and blacks. Within the axial skeleton 32% of cases were cranial, 32.8% spinal and 29.2% sacral. Young age (< 26 years; p = 0.0001) and female sex (p = 0.037) were associated with greater likelihood of cranial presentation. There was no overall increased risk for second primary cancers after chordoma. Median survival was 6.29 years; 5- and 10-year relative survival rates were 67.6% and 39.9%, respectively. Comparison with other bone sarcomas revealed racial disparities in incidence for the two developmental tumors, chordoma and Ewing's sarcoma.Conclusions: This study provides new data regarding incidence and survival patterns of chordoma in the US. Additional epidemiologic studies are required to elucidate the genetic and environmental determinants underlying this rare, distinctive neoplasm.