Ferrari Andrea, Orbach Daniel, Sparber-Sauer Monika, Walterhouse David O, Pajtler Kristian W, Meyer William H, Spunt Sheri L, Weiss Aaron R
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), PSL University, Institut Curie, Paris, France.
Eur J Cancer. 2022 Jul;169:10-19. doi: 10.1016/j.ejca.2022.03.028. Epub 2022 Apr 28.
The current article focuses on non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), the heterogeneous group of mesenchymal tumours different from rhabdomyosarcoma that may affect children and adolescents, with clinical behaviour varying from relatively benign to highly malignant. This review represents the effort of the international scientific paediatric community within the context of the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT), a project founded by the leadership of three large cooperative groups - Children's Oncology Group, Cooperative Weichteilsarkom Studiengruppe and European paediatric Soft tissue sarcoma Study Group - with the main goal to pool expertise and resources on a broader international level in order to improve knowledge of soft tissue sarcomas of children, adolescents and young adults. This article describes the current standard treatment approach in NRSTS, with a focus on the controversies and challenges in the management of these tumours. Developing research projects and clinical protocols for NRSTS has always been challenging, supporting the need to develop international integrated prospective dedicated programs, including paediatric NRSTS experts together with the adult sarcoma community. INSTRuCT provides a unique mechanism to increase international collaboration by agreeing on a common language, developing consensus standards to guide diagnosis and treatment, comparing clinical trial results across cooperative groups, and through a shared data dictionary providing answers to questions that can only be addressed by a larger data set.
本文聚焦于非横纹肌肉瘤软组织肉瘤（NRSTS），这是一组异质性的间充质肿瘤，与横纹肌肉瘤不同，可影响儿童和青少年，其临床行为从相对良性到高度恶性不等。本综述是国际儿科科学界在国际软组织肉瘤联盟（INSTRuCT）背景下所做的努力，该项目由三个大型合作组——儿童肿瘤研究组、软组织肉瘤合作研究组和欧洲儿科软组织肉瘤研究组牵头成立，主要目标是在更广泛的国际层面汇聚专业知识和资源，以增进对儿童、青少年和青年软组织肉瘤的了解。本文描述了NRSTS目前的标准治疗方法，重点关注这些肿瘤治疗中的争议和挑战。为NRSTS制定研究项目和临床方案一直具有挑战性，这支持了开展国际综合前瞻性专项计划的必要性，该计划需纳入儿科NRSTS专家以及成人肉瘤领域的专家。INSTRuCT提供了一种独特的机制，通过商定共同语言、制定指导诊断和治疗的共识标准、比较各合作组的临床试验结果，以及通过共享数据字典来回答只有更大数据集才能解决的问题，从而加强国际合作。
