Stork Theresa, Ranft Andreas, Aigner Clemens, Jurgens Heribert, Ladenstein Ruth L, Timmermann Beate, Van den Berg Henk, Dirksen Uta, Collaud Stéphane
Department of Thoracic Surgery, Ruhrlandklinik, University of Duisburg-Essen, 45239 Essen, Germany.
Department of Thoracic Surgery, Cologne-Merheim Hospital, University of Witten/Herdecke, 51109 Cologne, Germany.
Cancers (Basel). 2025 Jan 2;17(1):118. doi: 10.3390/cancers17010118.
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.
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.
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, = 3 (33%) patients had synchronous metastases. All patients underwent multiagent chemotherapy. Local therapy for non-metastatic patients was surgery ( = 2, 22%), surgery and radiotherapy ( = 2, 22%) or radiotherapy alone ( = 2, 22%). Surgery consisted of extended resections in most patients ( = 3, 33%). Five-year survival for the whole cohort was 64%. Apart from one patient who was lost to follow-up, all patients ( = 4) who had undergone surgery were alive at the end of follow-up.
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.
纵隔尤因肉瘤（EWS）极为罕见，文献中仅有少数病例报道。我们旨在更好地了解原发性纵隔EWS，描述在两项国际多中心前瞻性随机EWS试验中接受治疗的患者情况。
从EURO - E.W.I.N.G.99（ClinicalTrials.gov标识符：NCT00020566）和EWING 2008（ClinicalTrials.gov标识符：NCT00987636）试验的数据库中检索原发性纵隔EWS患者的数据。分析患者和治疗特征。
在2969例EWS患者中，9例（0.3%）患有原发性纵隔EWS。诊断时的中位年龄为30.5岁（4至49岁）。诊断时，3例（33%）患者有同步转移。所有患者均接受了多药化疗。非转移性患者的局部治疗为手术（2例，22%）、手术加放疗（2例，22%）或单纯放疗（2例，22%）。大多数患者（3例，33%）的手术为扩大切除术。整个队列的5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。
原发性纵隔EWS极为罕见，在EWS中的患病率为0.3%。与所有组织学类型的原发性纵隔肉瘤相比，5年生存率良好，且与不同起源的EWS一致。
