Schneiderman Brian A, Kliethermes Stephanie A, Nystrom Lukas M
Harbor-UCLA Medical Center, Los Angeles, CA, USA.
Stritch School of Medicine, Loyola University Chicago, 2160 South First Avenue, Maywood, IL, 60153, USA.
Clin Orthop Relat Res. 2017 Mar;475(3):799-805. doi: 10.1007/s11999-016-4779-2.
Studies suggest that mesenchymal chondrosarcoma is associated with a poorer prognosis and a higher proportion of extraskeletal tumors than conventional chondrosarcoma. However, these investigations have been small heterogeneous cohorts, limiting analysis of prognostic factors.
QUESTIONS/PURPOSES: (1) What is the 5- and 10-year survival rate of patients diagnosed with mesenchymal chondrosarcoma? (2) What is the effect of demographic and tumor characteristics on survival in patients with mesenchymal chondrosarcoma?
The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all patients diagnosed with mesenchymal chondrosarcoma from 1973 to 2011. SEER reports survival data on over 8.2 million patients with cancer and has attained 98% completeness in reporting. Using variables within the database, this study designated each patient's tumor as skeletal or extraskeletal and cranial, axial, or appendicular, respectively. Overall survival (OS) was determined for the entire series as well as each group. Median survival was calculated using Kaplan-Meier methods. Cox proportional hazards regression was used to determine whether demographic and tumor variables affected survival. Two hundred five patients with mesenchymal chondrosarcoma were identified, including 82 (40%) skeletal and 123 (60%) extraskeletal.
OS for the entire series was 51% (95% confidence interval [CI], 43%-58%) and 43% (95% CI, 35%-51%) at 5 and 10 years, respectively. No difference in OS was detected between extraskeletal and skeletal tumors. Kaplan-Meier analyses showed OS was worse for tumors in axial locations compared with appendicular and cranial locations. Appendicular tumors demonstrated an OS of 50% (95% CI, 36%-63%) at 5 years and 39% (95% CI, 26%-52%) at 10 years. OS for axial tumors was 37% (95% CI, 25%-49%) and 31% (95% CI, 20%-43%), whereas it was 74% (95% CI, 59%-84%) and 67% (95% CI, 50%-79%) for cranial tumors at 5 and 10 years, respectively. When controlling for age, sex, tumor origin, and tumor location, the presence of metastasis (hazard ratio [HR], 12.38; 95% CI, 5.75-26.65; p < 0.001) and 1-cm size increase (HR, 1.16; 95% CI, 1.09-1.23; p < 0.001) were both independently associated with an increased risk of death. Tumor location showed different behaviors depending on patient age. In comparison to cranial tumors at age 20 years, the HR was 5.56 (95% CI, 1.47-21.05; p = 0.01) for axial tumors and 6.26 (95% CI, 1.54-25.42; p = 0.01) for appendicular tumors. At age 60 years, those ratios were 0.10 (95% CI, 0.02-0.55; p = 0.01) and 0.14 (95% CI, 0.04-0.58; p = 0.01), respectively.
Our data suggest that extraskeletal tumors are more common than previously reported; however, this factor does not have clear prognostic value. Presence of metastatic disease and increased tumor size are the main predictors of poor survival outcome. Cranial tumors appear to have a different clinical behavior with our data suggesting better overall survival in young patients (compared with axial and appendicular locations) and a worse survival outcome in older patients.
Level IV, prognostic study.
研究表明，与传统软骨肉瘤相比，间叶性软骨肉瘤的预后较差，且骨外肿瘤的比例更高。然而，这些研究均为小型异质性队列研究，限制了对预后因素的分析。
问题/目的：（1）诊断为间叶性软骨肉瘤的患者5年和10年生存率是多少？（2）人口统计学和肿瘤特征对间叶性软骨肉瘤患者生存的影响如何？
使用监测、流行病学和最终结果（SEER）数据库，识别1973年至2011年间所有诊断为间叶性软骨肉瘤的患者。SEER报告了超过820万癌症患者的生存数据，报告完整性达到98%。利用数据库中的变量，本研究将每位患者的肿瘤分别指定为骨骼或骨外肿瘤，以及头颅、轴向或附属部位肿瘤。确定了整个队列以及每个组的总生存期（OS）。使用Kaplan-Meier方法计算中位生存期。采用Cox比例风险回归分析来确定人口统计学和肿瘤变量是否影响生存。共识别出205例间叶性软骨肉瘤患者，其中82例（40%）为骨骼肿瘤，123例（60%）为骨外肿瘤。
整个队列的5年和10年OS分别为51%（95%置信区间[CI]，43%-58%）和43%（95%CI，35%-51%）。骨外肿瘤和骨骼肿瘤之间未检测到OS差异。Kaplan-Meier分析显示，与附属和头颅部位的肿瘤相比，轴向部位的肿瘤OS更差。附属部位肿瘤的5年OS为50%（95%CI，36%-63%），10年OS为39%（95%CI，26%-52%）。轴向肿瘤的5年和10年OS分别为37%（95%CI，25%-49%）和31%（95%CI，20%-43%），而头颅肿瘤的5年和10年OS分别为74%（95%CI，59%-84%）和67%（95%CI，50%-79%）。在控制年龄、性别、肿瘤起源和肿瘤部位后，转移的存在（风险比[HR]，12.38；95%CI，5.75-26.65；p<0.001）和肿瘤大小增加1cm（HR，1.16；95%CI，1.09-1.23；p<0.001）均与死亡风险增加独立相关。肿瘤部位根据患者年龄表现出不同的行为。与20岁时的头颅肿瘤相比，轴向肿瘤的HR为5.56（95%CI，1.47-21.05；p=0.01），附属部位肿瘤的HR为6.26（95%CI，1.54-25.42；p=0.01）。在60岁时，这些比率分别为0.10（95%CI，0.02-0.55；p=0.01）和0.14（95%CI，0.04-0.58；p=0.01）。
我们的数据表明，骨外肿瘤比先前报道的更常见；然而，这一因素没有明确的预后价值。转移性疾病的存在和肿瘤大小增加是生存结果不佳的主要预测因素。头颅肿瘤似乎具有不同的临床行为，我们的数据表明年轻患者（与轴向和附属部位相比）的总体生存率更好，而老年患者的生存结果更差。
IV级，预后研究。
