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复发性横纹肌肉瘤:欧洲儿科软组织肉瘤研究组(EpSSG)的治疗建议

Relapsed rhabdomyosarcoma: treatment recommendations from the European pediatric soft tissue sarcoma study group (EpSSG).

作者信息

Defachelles A S, Breunis W B, Casanova M, Minard-Collin V, Hjadun R, Wasti A, Dávila Fajardo R, Terwisscha van Scheltinga S, Heenen D, Heinz A T, Sparber-Sauer M, Merks J H M, Chisholm J C, Gatz S A

机构信息

Department of Pediatric and AYA Oncology, Oscar Lambret Center, Lille, France.

Department of Oncology, University Children's Hospital Zürich, Zürich, Switzerland.

出版信息

Br J Cancer. 2025 Sep 5. doi: 10.1038/s41416-025-03130-1.

Abstract

At least one-third of patients with localized rhabdomyosarcoma (RMS) and 60-70% of patients with metastatic RMS experience progressive disease or relapse. Following relapse, outcomes generally remain poor with limited treatment options and a high risk of subsequent recurrence. Optimal treatment requires a multidisciplinary approach incorporating chemotherapy with local control. Given the complexity of managing relapsed RMS and the challenges in developing effective treatment strategies, we aim to present clear and practical recommendations on the management of these patients across Europe. These recommendations were developed collaboratively by a group of pediatric and adolescent sarcoma experts from the European paediatric Soft Tissue Sarcoma Study Group. A careful review of the literature was performed to ensure that wherever possible recommendations are supported by the results of clinical trials or substantive retrospective reports. Such recommendations provide a standardized approach to managing relapsed cases, improving patient outcomes and offering a framework for clinicians to make informed decisions.

摘要

至少三分之一的局限性横纹肌肉瘤(RMS)患者以及60%-70%的转移性RMS患者会出现疾病进展或复发。复发后,由于治疗选择有限且后续复发风险高,总体预后通常较差。最佳治疗需要采用多学科方法,将化疗与局部控制相结合。鉴于复发性RMS管理的复杂性以及制定有效治疗策略的挑战,我们旨在为欧洲各地这些患者的管理提出明确且实用的建议。这些建议由欧洲儿科软组织肉瘤研究组的一组儿科和青少年肉瘤专家共同制定。对文献进行了仔细审查,以确保建议尽可能得到临床试验结果或大量回顾性报告的支持。此类建议为复发病例的管理提供了标准化方法,改善了患者预后,并为临床医生做出明智决策提供了框架。

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