尤文肉瘤（ES）是一种骨和软组织恶性肿瘤，最常见于儿童、青少年和年轻人。对于复发/难治性 ES (RR-ES) 的治疗仍存在争议。作者利用了国家尤文肉瘤肿瘤委员会汇集的专业知识，该委员会是一个多学科虚拟肿瘤委员会，每月开会讨论具有挑战性的 ES 病例。在本次综述中，他们重点关注适用于 RR-ES 患者管理的精选主题。涵盖的具体主题包括此类患者的初步治疗方法和护理目标的讨论、分子检测的作用、化疗方案和需要考虑的新药物、维持治疗的作用以及自体干细胞高剂量化疗的使用细胞救援。引用的数据通常仅限于亚组分析和/或从多个来源编译。尽管无意取代治疗医生的临床判断，但这些指南旨在为临床医生提供支持，并为 RR-ES 患者的管理提供一些明确性和建议。通俗语言摘要：尤文肉瘤 (ES) 是一种骨和软组织癌症，最常发生于青少年和年轻人。本文借鉴了国家尤文肉瘤肿瘤委员会的经验，该委员会是一个多机构、多学科的虚拟肿瘤委员会，每月举行一次会议，讨论具有挑战性的 ES 病例，并解决与复发 ES 患者治疗相关的问题。虽然无意取代治疗医生的临床判断并且受到现有数据的限制，但这些共识建议将为治疗患有这种具有挑战性的恶性肿瘤的患者的临床医生提供支持，当这种恶性肿瘤复发时会变得更加困难。© 2024 作者。 《癌症》由 Wiley periodicals LLC 代表美国癌症协会出版。

Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue that most often occurs in children, adolescents, and young adults. Debate and controversy remain in the management of relapsed/refractory ES (RR-ES). The authors leveraged the expertise assembled by the National Ewing Sarcoma Tumor Board, a multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES. In this review, they focus on select topics that apply to the management of patients with RR-ES. The specific topics covered include the initial approach of such patients and discussion of the goals of care, the role of molecular testing, chemotherapy regimens and novel agents to consider, the role of maintenance therapy, and the use of high-dose chemotherapy with autologous stem cell rescue. The data referenced are often limited to subgroup analyses and/or compiled from multiple sources. Although not intended to replace the clinical judgement of treating physicians, these guidelines are intended to support clinicians and provide some clarity and recommendations for the management of patients with RR-ES. PLAIN LANGUAGE SUMMARY: Ewing sarcoma (ES) is a bone and soft tissue cancer that most often occurs in teenagers and young adults. This article uses the experience of the National Ewing Sarcoma Tumor Board, a multi-institution, multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES and to address questions related to the treatment of patients with relapsed ES. Although not intended to replace the clinical judgement of treating physicians and limited by available data, these consensus recommendations will support clinicians who treat patients with this challenging malignancy, made even more difficult when it recurs.© 2024 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.