Chen Jun, Li Mengxue, Zheng Yifeng, Zheng Lei, Fan Fanfan, Wang Yu
Department of Neurosurgery, Tongji Medical College, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China.
Department of Ultrasonics, The 991th Hospital of the Joint Logistics Support Unit of the Chinese People's Liberation Army, Xiangyang, China.
Front Oncol. 2019 Jun 25;9:555. doi: 10.3389/fonc.2019.00555. eCollection 2019.
Primary spinal Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare, and the current understanding of these tumors is poor. The authors aimed to illustrate the clinical characteristics of primary spinal ES/pPNETs and to discuss prognostic factors by survival analysis. A total of 40 patients who were pathologically diagnosed with primary spinal ES/pPNETs between 2000 and 2018 were enrolled in this study. Progression-free survival (PFS) and overall survival (OS) were estimated by the Kaplan-Meier method to identify potential prognostic factors. Factors of ≤ 0.1 in the Log-rank tests were subjected to multivariate analysis by Cox regression analysis. The mean follow-up period was 23.8 (range, 2-93) months, and 24 (60.0%) patients had local recurrence and 11 (27.5%) patients had distant metastasis. The 1-, 2-, and 5-year PFS rates were 57.7, 30.4, and 9.5%, respectively. The 1-, 2-, and 5-year OS rates were 74.8, 50.7, and 12.2%, respectively. The univariate analysis suggested that resection mode, postoperative Frankel score, adjuvant chemotherapy and adjuvant radiotherapy were potential prognostic factors for OS and PFS. However, after these factors were subjected to multivariate analyses, only adjuvant radiotherapy and resection mode remained as independent prognostic factors. Total en bloc resection can significantly improve PFS for primary spinal ES/pPNETs and adjuvant radiotherapy was a favorable factor for PFS. Total en bloc resection and adjuvant radiotherapy considerably improve OS for patients with primary spinal ES/pPNETs.
原发性脊柱尤文肉瘤（ES）/外周原始神经外胚层肿瘤（pPNETs）极为罕见，目前对这些肿瘤的了解甚少。作者旨在阐述原发性脊柱ES/pPNETs的临床特征，并通过生存分析探讨预后因素。本研究纳入了2000年至2018年间40例经病理诊断为原发性脊柱ES/pPNETs的患者。采用Kaplan-Meier法估计无进展生存期（PFS）和总生存期（OS），以确定潜在的预后因素。对数秩检验中P≤0.1的因素通过Cox回归分析进行多因素分析。平均随访时间为23.8（范围2 - 93）个月，24例（60.0%）患者出现局部复发，11例（27.5%）患者出现远处转移。1年、2年和5年的PFS率分别为57.7%、30.4%和9.5%。1年、2年和5年的OS率分别为74.8%、50.7%和12.2%。单因素分析表明，切除方式、术后Frankel评分、辅助化疗和辅助放疗是OS和PFS的潜在预后因素。然而，在对这些因素进行多因素分析后，只有辅助放疗和切除方式仍然是独立的预后因素。整块切除可显著提高原发性脊柱ES/pPNETs的PFS，辅助放疗是PFS的有利因素。整块切除和辅助放疗可显著提高原发性脊柱ES/pPNETs患者的OS。
