Department of Pediatrics, School of Medicine, University of California, San Francisco, California 94143-0106, USA.
Pediatr Blood Cancer. 2012 Oct;59(4):617-20. doi: 10.1002/pbc.24053. Epub 2011 Dec 19.
A minority of patients with Ewing sarcoma present with regional lymph node involvement. We investigated if patient characteristics and outcomes differ between patients with Ewing sarcoma with and without regional node involvement.
Patients <40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor (PNET) reported to the SEER database from 1973 to 2008 were evaluated based on the presence (n = 91) or absence (n = 1,361) of regional node involvement. Patient characteristics were analyzed using Fisher exact tests. Overall survival was estimated by Kaplan-Meier methods and evaluated using log-rank tests and Cox models.
Patients with regional node involvement were more likely to have extraskeletal primary tumors (65.9% vs. 31.2%; P < 0.001) and axial tumors (71.1% vs. 59.6%; P = 0.03) compared to patients without regional node involvement. The incidence of regional node involvement was 12.4% for patients with extraskeletal primary tumors compared to 3.2% for patients with skeletal tumors. Five-year overall survival from diagnosis was inferior for patients with regional node involvement compared to those without regional node involvement (45.9% vs. 60.3%; P < 0.001). On multivariate analysis, regional node involvement was predictive of inferior overall survival independent of age, metastatic status, tumor site, and soft tissue origin (hazard ratio 1.59; 95% CI 1.16-2.19).
Patients with extraskeletal Ewing sarcoma should undergo evaluation for regional node involvement. If validated, our findings indicate that regional node involvement may be an independent adverse prognostic factor in Ewing sarcoma, and potentially useful in risk-stratifying patients with otherwise localized disease.
少数尤文肉瘤患者存在区域性淋巴结受累。我们研究了尤文肉瘤或外周原始神经外胚层肿瘤（PNET）患者中，有和无区域性淋巴结受累的患者特征和结局是否存在差异。
根据是否存在（n=91）或不存在（n=1361）区域性淋巴结受累，评估了 1973 年至 2008 年期间 SEER 数据库中年龄<40 岁的尤文肉瘤或外周原始神经外胚层肿瘤患者。使用 Fisher 确切检验分析患者特征。通过 Kaplan-Meier 方法估计总生存率，并使用对数秩检验和 Cox 模型进行评估。
与无区域性淋巴结受累的患者相比，有区域性淋巴结受累的患者更有可能有骨外原发性肿瘤（65.9% vs. 31.2%；P<0.001）和轴性肿瘤（71.1% vs. 59.6%；P=0.03）。与有骨外原发性肿瘤的患者相比，有骨原发性肿瘤的患者发生区域性淋巴结受累的发生率为 12.4%，而有骨原发性肿瘤的患者发生率为 3.2%。与无区域性淋巴结受累的患者相比，有区域性淋巴结受累的患者从诊断起的 5 年总生存率较低（45.9% vs. 60.3%；P<0.001）。多变量分析表明，区域淋巴结受累是独立于年龄、转移状态、肿瘤部位和软组织起源的总生存率不良的预测因素（风险比 1.59；95%置信区间 1.16-2.19）。
有骨外尤文肉瘤的患者应接受区域性淋巴结受累的评估。如果得到验证，我们的发现表明，区域淋巴结受累可能是尤文肉瘤的一个独立不良预后因素，并可能有助于对其他局部疾病患者进行风险分层。
