Imaging in rhabdomyosarcoma: a patient journey

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.

Automated summary

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents, although it is rare. It can occur in various parts of the body, with the head and neck, genitourinary tract, and extremities being the most common sites. The optimal approach to diagnosing and treating rhabdomyosarcoma involves a multidisciplinary team and multimodal treatment. Despite improvements in survival, further advancements in management are necessary as the 5-year overall survival rate varies from 35% to 100% depending on the disease and patient characteristics. In the journey of two patients with rhabdomyosarcoma, the role and impact of the pediatric radiologist and nuclear physician in the clinical setting are discussed, emphasizing the implementation of guidelines and providing up-to-date insights on all aspects of the disease, from diagnosis to follow-up.