Department of Orthopaedic Surgery, University of California, Irvine, Irvine, California, USA.
J Bone Joint Surg Am. 2012 Feb 15;94(4):356-63. doi: 10.2106/JBJS.J.01784.
Chordoma is the most common primary malignant tumor of the spine. It is extremely rare and has been studied primarily in single-institution case series. Using data from a large, population-based cancer registry, we designed the present study to examine the outcome for patients with chordoma and to determine relevant prognostic factors.
A retrospective analysis of the California Cancer Registry database was performed to identify patients with a diagnosis of chordoma in the years 1989 to 2007. Comparisons examined differences in demographics, disease characteristics, treatment, and survival. Survival analyses were performed with use of the Kaplan-Meier method with log-rank tests and Cox proportional hazards models.
Four hundred and nine patients with chordoma were identified; 257 (62.8%) were male and 152 (37.2%) were female. With regard to racial or ethnic distribution, 266 patients (65%) were white; ninety-three (22.7%), Hispanic; forty-three (10.5%), Asian or other; and seven (1.7%), black. The site of presentation was the head in 202 patients (49.4%), spine in 106 patients (25.9%), and pelvis and/or sacrum in 101 patients (24.7%). Hispanic race (p = 0.0002), younger age (less than forty years; p < 0.0001), and female sex (p = 0.009) were associated with cranial presentation, whereas older age (forty years or older; p < 0.0001) was associated with pelvic presentation. After adjustment for clinically relevant factors, a significantly decreased risk of death for chordoma-specific survival was seen for Hispanic race (hazard ratio = 0.51, 95% confidence interval [95% CI], 0.28 to 0.93; p = 0.03), high socioeconomic status (hazard ratio = 0.8, 95% CI, 0.67 to 0.95; p = 0.01), and local excision and/or debulking (hazard ratio = 0.38, 95% CI, 0.18 to 0.81; p = 0.01). Large tumor size was independently associated with an increased risk of death (hazard ratio = 2.05, 95% CI, 1.01 to 4.20; p = 0.048).
In this study, the survival of patients with chordoma was significantly better for those who were Hispanic and had a small tumor, high socioeconomic status, and surgical intervention.
脊索瘤是最常见的原发性脊柱恶性肿瘤。它非常罕见，主要在单机构病例系列中进行研究。利用来自大型人群癌症登记处的数据，我们设计了本研究，以检查脊索瘤患者的预后，并确定相关的预后因素。
对加利福尼亚癌症登记处数据库进行回顾性分析，以确定 1989 年至 2007 年间诊断为脊索瘤的患者。比较检查了人口统计学、疾病特征、治疗和生存方面的差异。使用 Kaplan-Meier 方法和对数秩检验以及 Cox 比例风险模型进行生存分析。
共确定了 409 例脊索瘤患者；257 例（62.8%）为男性，152 例（37.2%）为女性。在种族或民族分布方面，266 例（65%）为白人；93 例（22.7%）为西班牙裔；43 例（10.5%）为亚洲裔或其他；7 例（1.7%）为黑人。202 例（49.4%）患者首发于头部，106 例（25.9%）患者首发于脊柱，101 例（24.7%）患者首发于骨盆和/或骶骨。西班牙裔种族（p = 0.0002）、年龄较小（<40 岁；p < 0.0001）和女性（p = 0.009）与颅部首发相关，而年龄较大（≥40 岁；p < 0.0001）与骨盆首发相关。在调整了临床相关因素后，西班牙裔种族的脊索瘤特异性生存率显著降低（风险比=0.51，95%置信区间[95%CI]，0.28 至 0.93；p = 0.03），高社会经济地位（风险比=0.8，95%CI，0.67 至 0.95；p = 0.01）和局部切除和/或肿瘤缩小（风险比=0.38，95%CI，0.18 至 0.81；p = 0.01）。肿瘤较大与死亡风险增加独立相关（风险比=2.05，95%CI，1.01 至 4.20；p = 0.048）。
在这项研究中，西班牙裔患者和肿瘤较小、社会经济地位较高以及接受手术干预的脊索瘤患者的生存率显著提高。
