Objective: Ewing sarcoma (EWS) of the mediastinum is extremely rare, with only a few cases reported in the literature. We aimed to gain a better understanding of primary mediastinal EWS, describing patients treated within two international, multicenter, prospective, randomized EWS trials. Methods: Data from patients with primary mediastinal EWS were retrieved from the database of the EURO-E.W.I.N.G.99 (ClinicalTrials.gov identifier: NCT00020566) and EWING 2008 (ClinicalTrials.gov identifier: NCT00987636) trials. Patient and treatment characteristics were analyzed. Results: Out of 2969 patients with EWS, 9 (0.3%) had primary mediastinal EWS. The median age at diagnosis was 30.5 years (4 to 49). At the time of diagnosis,n= 3 (33%) patients had synchronous metastases. All patients underwent multiagent chemotherapy. Local therapy for non-metastatic patients was surgery (n= 2, 22%), surgery and radiotherapy (n= 2, 22%) or radiotherapy alone (n= 2, 22%). Surgery consisted of extended resections in most patients (n= 3, 33%). Five-year survival for the whole cohort was 64%. Apart from one patient who was lost to follow-up, all patients (n= 4) who had undergone surgery were alive at the end of follow-up. Conclusions: Primary mediastinal ES is extremely rare, with a prevalence of 0.3% among EWS. Five-year survival was favorable compared to primary mediastinal sarcoma of all histologies and in line with EWS of different origin.
目的：纵隔尤文肉瘤（EWS）极为罕见，文献中仅报道过少数病例。本研究旨在深入了解原发性纵隔EWS，描述在两个国际性、多中心、前瞻性、随机EWS临床试验中接受治疗的患者情况。方法：从EURO-E.W.I.N.G.99（ClinicalTrials.gov标识符：NCT00020566）和EWING 2008（ClinicalTrials.gov标识符：NCT00987636）试验数据库中提取原发性纵隔EWS患者数据，分析患者特征及治疗情况。结果：在2969例EWS患者中，9例（0.3%）为原发性纵隔EWS。诊断时中位年龄为30.5岁（范围4-49岁）。确诊时3例（33%）患者存在同步转移。所有患者均接受多药联合化疗。非转移患者的局部治疗方式包括：单纯手术（2例，22%）、手术联合放疗（2例，22%）或单纯放疗（2例，22%）。手术患者中多数（3例，33%）接受扩大切除术。全队列五年生存率为64%。除1例失访患者外，所有接受手术的患者（4例）在随访结束时均存活。结论：原发性纵隔EWS极为罕见，在EWS中的患病率为0.3%。其五年生存率优于所有组织学类型的原发性纵隔肉瘤，且与其他原发部位的EWS生存率相当。
肿瘤(癌症)患者之家