Division of Otolaryngology, Department of Surgery, Yale University School of Medicine, New Haven, Connecticut, U.S.A.
Yale Cancer Center, New Haven, Connecticut, U.S.A.
Laryngoscope. 2020 Oct;130(10):2385-2392. doi: 10.1002/lary.28412. Epub 2019 Nov 27.
Ewing sarcoma of the head and neck (ESHN) is a rare malignancy for which limited data exists. Herein we examine anatomic distribution of ESHN, demographic characteristics, and multi-modal therapy as potential determinants in the overall survival (OS) of patients with ESHN.
A retrospective study of the National Cancer Database (NCDB) from 2004-2016 was performed. A multivariate Cox regression and univariate Kaplan-Meier survival analyses were conducted.
We identified 284 patients with ESHN eligible for demographic analysis and 223 for survival analysis. Approximately half (50.3%) of patients with ESHN were under the age of 18. Pediatric patients (<18) were less likely to present with distant metastases (≤8.0% vs. 9.5-19.0%, P = .006) and more likely to present with osseous disease (90.2% vs. 75.2%, P = .001) than adults. Adult age (HR: 2.727, 95% CI, 1.381-5.384, P = .004) and distant metastatic disease at the time of presentation (HR: 8.161, 95% CI, 2.922-22.790, P < .001) were independently associated with worse OS. The addition of local therapy (either surgery, radiotherapy, or both) to chemotherapy was not associated with improved survival when compared to treatment with chemotherapy alone.
Predictors of OS in patients with ESHN included age < 18 years and non-metastatic disease at the time of diagnosis. Tumor site of origin (osseous vs. extraosseous primary) or the addition of local therapy to chemotherapy had no impact on OS. Our analysis suggests that chemotherapy serves as the primary modality in treating ESHN, while further study of this rare malignancy is required to discern the utility of combined systemic and local therapy. Laryngoscope, 130:2385-2392, 2020.
头颈部尤文肉瘤（ESHN）是一种罕见的恶性肿瘤，目前相关数据有限。在此，我们研究 ESHN 的解剖分布、人口统计学特征以及多模式治疗作为潜在决定因素，以评估 ESHN 患者的总生存率（OS）。
对 2004 年至 2016 年国家癌症数据库（NCDB）进行回顾性研究。进行多变量 Cox 回归和单变量 Kaplan-Meier 生存分析。
我们确定了 284 例符合人口统计学分析标准的 ESHN 患者和 223 例符合生存分析标准的患者。约一半（50.3%）的 ESHN 患者年龄在 18 岁以下。儿科患者（<18 岁）发生远处转移的可能性较小（≤8.0%比 9.5-19.0%，P=0.006），而发生骨病的可能性较大（90.2%比 75.2%，P=0.001）。成人年龄（HR：2.727，95%CI，1.381-5.384，P=0.004）和就诊时的远处转移疾病（HR：8.161，95%CI，2.922-22.790，P<0.001）与较差的 OS 独立相关。与单独化疗相比，化疗联合局部治疗（手术、放疗或两者联合）并未改善生存。
影响 ESHN 患者 OS 的预测因素包括年龄<18 岁和诊断时无远处转移疾病。肿瘤起源部位（骨内 vs. 骨外原发）或化疗联合局部治疗对 OS 无影响。我们的分析表明，化疗是治疗 ESHN 的主要方式，而需要进一步研究这种罕见的恶性肿瘤，以确定联合全身和局部治疗的效果。《喉镜》，130：2385-2392，2020。
