Review

关于复发性尤文氏肉瘤系统治疗的共识建议：国家尤文氏肉瘤肿瘤委员会报告

Consensus recommendations for systemic therapies in the management of relapsed Ewing sarcoma: A report from the National Ewing Sarcoma Tumor Board

CANCER

影响因子:5.1

分区:医学1区 Top / 肿瘤学2区

发表日期:2024 Dec 01

作者: Ajay Gupta, Matthew S Dietz, Richard F Riedel, Aditi Dhir, Scott C Borinstein, Michael S Isakoff, Jamie M Aye, Nino Rainusso, Amy E Armstrong, Steven G DuBois, Lars M Wagner, Jeremy M Rosenblum, Sarah Cohen-Gogo, Catherine M Albert, Stacey Zahler, Rashmi Chugh, Matteo Trucco

DOI: 10.1002/cncr.35537

摘要

尤文氏肉瘤（ES）是一种起源于骨骼和软组织的恶性肿瘤，最常发生于儿童、青少年和年轻成人。关于复发/难治性ES（RR-ES）的管理仍存在争议。作者利用由国家尤文氏肉瘤肿瘤委员会（一个每月举行的多学科虚拟肿瘤会）汇集的专业知识，讨论具有挑战性的ES病例。本次综述聚焦于RR-ES患者管理的特定主题，包括初步治疗策略与护理目标的讨论、分子检测的作用、化疗方案及新型药物的考虑、维持治疗的作用以及高剂量化疗结合自体干细胞移植的使用。所引用的数据多局限于亚组分析和/或来自多个来源。尽管本指南不能替代治疗医生的临床判断，但旨在为临床医生提供支持，明确RR-ES患者管理的建议和指导。简明版：尤文氏肉瘤（ES）是一种多发于青少年和年轻成人的骨骼和软组织癌症。本文借助国家尤文氏肉瘤肿瘤委员会的经验——一个每月讨论疑难病例的多机构多学科虚拟肿瘤会，探讨与RR-ES患者治疗相关的关键问题。虽然不能取代临床医生的判断，且受限于现有数据，这些共识建议将帮助治疗此类复杂恶性肿瘤的临床医生，尤其是在复发时的治疗决策更加复杂。

Abstract

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.