Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.
Department of Radiology and Nuclear Medicine, University Medical Centre Utrecht, Utrecht, the Netherlands.
Pediatr Radiol. 2023 Apr;53(4):788-812. doi: 10.1007/s00247-023-05596-8. Epub 2023 Feb 27.
Rhabdomyosarcoma, although rare, is the most frequent soft tissue sarcoma in children and adolescents. It can present as a mass at nearly any site in the body, with most common presentations in the head and neck, genitourinary tract and extremities. The optimal diagnostic approach and management of rhabdomyosarcoma require a multidisciplinary team with multimodal treatment, including chemotherapy and local therapy. Survival has improved over the last decades; however, further improvement in management is essential with current 5-year overall survival ranging from 35% to 100%, depending on disease and patient characteristics. In the full patient journey, from diagnosis, staging, management to follow-up after therapy, the paediatric radiologist and nuclear physician are essential members of the multidisciplinary team. Recently, guidelines of the European paediatric Soft tissue sarcoma Study Group, the Cooperative Weichteilsarkom Studiengruppe and the Oncology Task Force of the European Society of Paediatric Radiology (ESPR), in an ongoing collaboration with the International Soft-Tissue Sarcoma Database Consortium, provided guidance for high-quality imaging. In this educational paper, given as a lecture during the 2022 postgraduate ESPR course, the multi-disciplinary team of our national paediatric oncology centre presents the journey of two patients with rhabdomyosarcoma and discusses the impact on and considerations for the clinical (paediatric) radiologist and nuclear physician. The key learning points of the guidelines and their implementation in clinical practice are highlighted and up-to-date insights provided for all aspects from clinical suspicion of rhabdomyosarcoma and its differential diagnosis, to biopsy, staging, risk stratification, treatment response assessment and follow-up.
横纹肌肉瘤虽然罕见,但却是儿童和青少年中最常见的软组织肉瘤。它可以在身体的几乎任何部位出现肿块,最常见的部位是头颈部、泌尿生殖系统和四肢。横纹肌肉瘤的最佳诊断方法和治疗需要一个多学科团队,采用多模式治疗,包括化疗和局部治疗。在过去几十年中,生存率有所提高;然而,随着目前 5 年总生存率从 35%到 100%不等,取决于疾病和患者特征,进一步改善管理至关重要。在完整的患者治疗过程中,从诊断、分期、治疗到治疗后随访,儿科放射科医生和核医学医生是多学科团队的重要成员。最近,欧洲儿科软组织肉瘤研究组、合作软组织肉瘤研究组和欧洲儿科放射学会(ESPR)肿瘤学工作组在与国际软组织肉瘤数据库联盟的持续合作中,为高质量成像提供了指导。在这篇教育论文中,作为 2022 年 ESPR 研究生课程的演讲,我们国家儿科肿瘤中心的多学科团队展示了两名横纹肌肉瘤患者的治疗过程,并讨论了对临床(儿科)放射科医生和核医学医生的影响和考虑。强调了指南的主要学习要点及其在临床实践中的实施,并提供了有关从横纹肌肉瘤的临床怀疑及其鉴别诊断到活检、分期、风险分层、治疗反应评估和随访等各个方面的最新见解。