Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.
Institute of Radiology Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin, Klinikum Stuttgart, Stuttgart, Germany.
Eur J Radiol. 2023 Sep;166:111012. doi: 10.1016/j.ejrad.2023.111012. Epub 2023 Jul 25.
Rhabdomyosarcoma is the most common soft-tissue neoplasm in the pediatric population. The survival of children with rhabdomyosarcoma has only marginally improved over the past 25 years and remains poor for those with metastatic disease. A significant challenge to advances in treatment of rhabdomyosarcoma is the relative rarity of this disease, necessitating years to complete clinical trials. Progress can be accelerated by international cooperation and sharing national experiences. This necessitates agreement on a common language to describe patient cohorts and consensus standards to guide diagnosis, treatment, and response assessment. These goals formed the premise for creating the INternational Soft Tissue saRcoma ConsorTium (INSTRuCT) in 2017. Multidisciplinary members of this consortium have since developed international consensus statements on the diagnosis, treatment, and management of pediatric soft-tissue sarcomas. Herein, members of the INSTRuCT Diagnostic Imaging Working Group present international consensus recommendations for imaging of patients with rhabdomyosarcoma at diagnosis, at staging, and during and after completion of therapy. The intent is to promote a standardized imaging approach to pediatric patients with this malignancy to create more-reliable comparisons of results of clinical trials internationally, thereby accelerating progress in managing rhabdomyosarcoma and improving survival.
横纹肌肉瘤是儿童中最常见的软组织肿瘤。过去 25 年来,横纹肌肉瘤患儿的生存率仅略有提高,转移性疾病患儿的生存率仍然较差。横纹肌肉瘤治疗进展的一个重大挑战是这种疾病相对罕见,需要数年时间才能完成临床试验。通过国际合作和分享国家经验可以加速进展。这需要就描述患者队列的通用语言以及指导诊断、治疗和反应评估的共识标准达成一致。这些目标构成了 2017 年创建国际软组织肉瘤联合会(INSTRuCT)的前提。该联合会的多学科成员此后就儿科软组织肉瘤的诊断、治疗和管理制定了国际共识声明。在此,INSTRuCT 诊断成像工作组的成员提出了横纹肌肉瘤患者在诊断、分期以及治疗期间和治疗后进行影像学检查的国际共识建议。目的是为患有这种恶性肿瘤的儿科患者制定标准化的影像学方法,以便在国际上更可靠地比较临床试验结果,从而加速横纹肌肉瘤的治疗进展并提高生存率。