尤文肉瘤家族肿瘤 (ESFT) 是一组罕见的恶性肿瘤，其中大约三分之一的病例表现出转移性扩散，特别是当骨和/或骨髓 (BM) 受累时，会影响预后。原发性肺外转移性 ESFT 通常需要强化治疗方法。准确的分期在临床决策中发挥着关键作用，氟-18-氟脱氧葡萄糖-正电子发射断层扫描/计算机断层扫描（PET/CT）目前作为评估 ESFT BM 范围的非侵入性方式。在法国 II 期 COMBINAIR3 (NCT03011528) 研究评估了肺外 ESFT 转移患者的综合方法。我们前瞻性地将 PET/CT 与 BM 抽吸和活检 (BMAB) 分析对接受初始分期的患者进行比较。在分析的 42 名患者中（中位年龄 14 岁，男女比例 2:1），45% 患有盆腔原发性病变肿瘤，83% 在诊断时有骨/BM 受累。我们的研究结果表明，PET/CT 在检测初始 BM 受累方面具有 100% 的特异性和 83.3% 的敏感性。总体而言，PET/CT对92.8%的患者进行了正确分类，在确定骨受累的患者中达到了100%的准确率，从而超越了标准BMAB。这些结果表明，在高危ESFT患者的初始分期中常规使用BMAB是可以的省略，推广 PET/CT 作为一种非侵入性替代方案，从而提高 ESFT 管理中的分期准确性和治疗决策。© 2024。作者，获得 Springer Nature Limited 的独家许可。

The Ewing Sarcoma Family of Tumors (ESFT) constitutes a group of rare malignancies, wherein approximately one-third of cases exhibit metastatic spread, particularly impacting prognosis when bone and/or bone marrow (BM) are involved. Primary extra-pulmonary metastatic ESFT often necessitates intensified therapeutic approaches. Accurate staging plays a pivotal role in clinical decision-making, with fluorine-18-fluorodeoxyglucose-positron emission tomography/computed tomography (PET/CT) currently serving as a non-invasive modality for assessing ESFT's BM extent.In the French phase II COMBINAIR3 (NCT03011528) study, a comprehensive approach for patients with extra-pulmonary ESFT metastasis was evaluated. We prospectively compared the efficacy of PET/CT to BM aspiration and biopsy (BMAB) analysis in patients undergoing initial staging.Among the 42 patients analyzed (median age 14 y, 2:1 male/female ratio), 45% presented with pelvic primary tumors and 83% had bone/BM involvement at diagnosis. Our findings showed PET/CT had 100% specificity and 83.3% sensitivity in detecting initial BM involvement. Overall, PET/CT correctly classified 92.8% of patients, reaching 100% accuracy in patients identified with bone involvement, thus surpassing the standard BMAB.These results suggest that the conventional use of BMAB in the initial staging of high-risk ESFT patients can be omitted, promoting PET/CT as a non-invasive alternative, thus improving staging accuracy and treatment decisions in ESFT management.© 2024. The Author(s), under exclusive licence to Springer Nature Limited.