关于复发肉瘤管理中系统疗法的共识建议：国家尤因肉瘤肿瘤委员会的报告

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:5.10000

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

摘要

尤因肉瘤（ES）是一种骨和软组织的恶性肿瘤，最常发生在儿童，青少年和年轻人中。辩论和争议仍然存在于复发/耐火性ES（RR-ES）的管理中。作者利用了国家尤因肉瘤肿瘤委员会组装的专业知识，该专业知识是一个多学科虚拟肿瘤委员会，每月开会，讨论ES的挑战性案例。在这篇综述中，他们着重于适用于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方法以及有关护理目标的讨论，分子测试的作用，化学疗法方案和新颖的药物，维持疗法的作用以及使用自体干细胞救援的高剂量化学疗法。引用的数据通常仅限于子组分析和/或从多个来源编译的数据。尽管不打算取代治疗医师的临床判断，但这些准则旨在支持临床医生，并为患有RR-ES患者的管理提供一些清晰和建议。简单的语言摘要：Ewing肉瘤（ES）是一种骨骼和软组织癌，最常发生在青少年和年轻人中。本文使用了国家尤因肉瘤肿瘤委员会的经验，该肿瘤委员会是一个多学科的多学科虚拟肿瘤委员会，每月符合每月讨论ES的挑战性案例，并解决与复发ES患者治疗有关的问题。尽管不打算取代治疗医生的临床判断并受到可用数据的限制，但这些共识建议将支持对治疗这种挑战性恶性肿瘤患者的临床医生，在复发时使其变得更加困难。

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.