Department of Nuclear Medicine, Curie Institute, Paris, France.
Department of Pediatric Oncology, Institute for Paediatric Haematology and Oncology, Léon Bérard Center, Lyon, France.
Br J Cancer. 2024 Nov;131(10):1605-1612. doi: 10.1038/s41416-024-02864-8. Epub 2024 Oct 8.
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.
尤文氏肉瘤家族肿瘤(ESFT)构成一组罕见的恶性肿瘤,其中约三分之一的病例存在转移性扩散,尤其是当涉及骨骼和/或骨髓(BM)时,会对预后产生影响。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起着关键作用,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)目前是评估 ESFT BM 范围的一种非侵入性方法。
在法国 II 期 COMBINAIR3 研究(NCT03011528)中,评估了针对肺外 ESFT 转移患者的综合方法。我们前瞻性比较了在初始分期中进行的 PET/CT 与骨髓抽吸和活检(BMAB)分析在患者中的疗效。
在分析的 42 名患者中(中位年龄 14 岁,男女比例为 2:1),45%的患者有骨盆原发肿瘤,83%的患者在诊断时存在骨骼/BM 受累。我们的研究结果表明,PET/CT 在检测初始 BM 受累时具有 100%的特异性和 83.3%的敏感性。总体而言,PET/CT 正确分类了 92.8%的患者,在有骨骼受累的患者中达到了 100%的准确性,因此优于标准的 BMAB。
这些结果表明,在高危 ESFT 患者的初始分期中可以省略常规使用 BMAB,促进 PET/CT 作为一种非侵入性替代方法,从而提高 ESFT 管理中的分期准确性和治疗决策。