Departments of Radiation Oncology.
Orthopedic Surgery.
Am J Clin Oncol. 2019 Oct;42(10):744-748. doi: 10.1097/COC.0000000000000590.
We evaluated our experience treating patients with localized extraskeletal myxoid chondrosarcomas (EMCs) to evaluate outcomes and relapse rates in order to better inform treatment decisions for these rare soft tissue sarcomas.
We reviewed the records of 41 consecutive patients with localized EMC treated at our institution from 1990 to 2016. Most patients (n=33, 80%) received combined modality therapy with surgery and radiation therapy, whereas only 8 (20%) underwent surgery alone. The Kaplan-Meier method was used to estimate rates of overall survival, disease-specific survival, local control (LC), and distant metastatic-free survival (DMFS).
Median follow-up time was 94 months (range, 8 to 316). The 10-year LC, DMFS, disease-specific survival, and overall survival rates were 90%, 69%, 85%, and 66%, respectively. There were 5 patients (12%) with local relapse at a median time of 75 months (range, 13 to 176). On univariate analysis, the only significant factor associated with poorer LC was the use of surgery alone (10 y LC, 63% vs. 100% for combined modality therapy, P=0.004), which remained the only factor also significant on the multivariable analysis (P=0.02; hazard ratio [HR], 12.7; 95% confidence interval [CI], 1.4-115.3). In total, 13 patients (32%) developed distant metastatic at a median time of 28 months (range, 3 to 154). Interestingly, local recurrence was the only factor associated with poorer DMFS on multivariate analysis (P=0.04; HR, 3.9; 95% CI, 1.1-14.7).
For patients with EMC, surgery alone was associated with a higher risk of local recurrence. Therefore, we recommend optimal local therapeutic strategies upfront with both surgery and radiation therapy to reduce the risk of local and ultimately distant recurrence.
我们评估了治疗局限性骨外黏液样软骨肉瘤（EMC）患者的经验，以评估结局和复发率，从而更好地为这些罕见的软组织肉瘤的治疗决策提供信息。
我们回顾了 1990 年至 2016 年在我院治疗的 41 例局限性 EMC 患者的病历。大多数患者（n=33，80%）接受了手术联合放化疗的综合治疗，而仅有 8 例（20%）仅接受了手术治疗。采用 Kaplan-Meier 法估计总生存率、疾病特异性生存率、局部控制率（LC）和远处无转移生存率（DMFS）。
中位随访时间为 94 个月（范围，8 至 316 个月）。10 年 LC、DMFS、疾病特异性生存率和总生存率分别为 90%、69%、85%和 66%。有 5 例（12%）患者在中位时间 75 个月（范围，13 至 176 个月）时出现局部复发。单因素分析显示，与较差的 LC 相关的唯一显著因素是单独使用手术（10 年 LC，单独手术组为 63%，综合治疗组为 100%，P=0.004），多因素分析也显示这是唯一显著的因素（P=0.02；风险比[HR]，12.7；95%置信区间[CI]，1.4-115.3）。共有 13 例（32%）患者在中位时间 28 个月（范围，3 至 154 个月）时发生远处转移。有趣的是，多因素分析显示，局部复发是 DMFS 较差的唯一相关因素（P=0.04；HR，3.9；95% CI，1.1-14.7）。
对于 EMC 患者，单独手术与局部复发风险增加相关。因此，我们建议在初始治疗中采用手术联合放疗的最佳局部治疗策略，以降低局部和最终远处复发的风险。
