TY - JOUR
T1 - Imaging in rhabdomyosarcoma
T2 - a patient journey
AU - de Vries, Isabelle S. A.
AU - van Ewijk, Roelof
AU - Adriaansen, Laura M. E.
AU - Bohte, Anneloes E.
AU - Braat, Arthur J. A. T.
AU - Fajardo, Raquel D. vila
AU - Hiemcke-Jiwa, Laura S.
AU - Hol, Marinka L. F.
AU - ter Horst, Simone A. J.
AU - de Keizer, Bart
AU - Knops, Rutger R. G.
AU - Meister, Michael T.
AU - Schoot, Reineke A.
AU - Smeele, Ludi E.
AU - van Scheltinga, Sheila Terwisscha
AU - Vaarwerk, Bas
AU - Merks, Johannes H. M.
AU - van Rijn, Rick R.
N1 - Funding Information: I.S.A. de Vries and R. van Ewijk are joint first author. J.H.M. Merks and R.R. van Rijn are joint last author. The authors would like to thank Hidde van Nieuwenhoven for drawing the graph depicted in Figure 2 and R.W.J. Meijers for his assistance with Figure 9. Publisher Copyright: © 2023, The Author(s).
PY - 2023/4
Y1 - 2023/4
N2 - 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.
AB - 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.
KW - Adolescent
KW - Child
KW - Medical oncology
KW - Radiology
KW - Rhabdomyosarcoma
UR - http://www.scopus.com/inward/record.url?scp=85148899177&partnerID=8YFLogxK
U2 - https://doi.org/10.1007/s00247-023-05596-8
DO - https://doi.org/10.1007/s00247-023-05596-8
M3 - Article
C2 - 36843091
SN - 0301-0449
VL - 53
SP - 788
EP - 812
JO - Pediatric Radiology
JF - Pediatric Radiology
IS - 4
ER -