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Published in: Pediatric Radiology 4/2023

Open Access 27-02-2023 | Rhabdomyosarcoma | ESPR

Imaging in rhabdomyosarcoma: a patient journey

Authors: Isabelle S. A. de Vries, Roelof van Ewijk, Laura M. E. Adriaansen, Anneloes E. Bohte, Arthur J. A. T. Braat, Raquel Dávila Fajardo, Laura S. Hiemcke-Jiwa, Marinka L. F. Hol, Simone A. J. ter Horst, Bart de Keizer, Rutger R. G. Knops, Michael T. Meister, Reineke A. Schoot, Ludi E. Smeele, Sheila Terwisscha van Scheltinga, Bas Vaarwerk, Johannes H. M. Merks, Rick R. van Rijn

Published in: Pediatric Radiology | Issue 4/2023

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Abstract

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.
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Literature
2.
go back to reference Wyatt KD, Birz S, Hawkins DS, Minard‐Colin V, Rodeberg DA, Sparber‐Sauer M, Bisogno G, Koscielniak E, De Salvo GL, Ebinger M, Merks JH (2022) Creating a data commons: the international soft tissue sarcoma consortium (INSTRuCT). Pediatr Blood Cancer 69(11):e29924. https://doi.org/10.1002/pbc.29924 Wyatt KD, Birz S, Hawkins DS, Minard‐Colin V, Rodeberg DA, Sparber‐Sauer M, Bisogno G, Koscielniak E, De Salvo GL, Ebinger M, Merks JH (2022) Creating a data commons: the international soft tissue sarcoma consortium (INSTRuCT). Pediatr Blood Cancer 69(11):e29924. https://​doi.​org/​10.​1002/​pbc.​29924
10.
go back to reference Shuman C, Beckwith BJ, Weksberg R (2000) Beckwith-Wiedemann Syndrome Synonym: Wiedemann-Beckwith Syndrome. [Updated 2016 Aug 11]. In: Adam MP, Everman DB, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2022 Shuman C, Beckwith BJ, Weksberg R (2000) Beckwith-Wiedemann Syndrome Synonym: Wiedemann-Beckwith Syndrome. [Updated 2016 Aug 11]. In: Adam MP, Everman DB, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2022
14.
go back to reference Hibbitts E, Bradley JA, Rudzinski ER, et al (2019) Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors : a report from the Children ’ s Oncology Group. 6437–6448. https://doi.org/10.1002/cam4.2504 Hibbitts E, Bradley JA, Rudzinski ER, et al (2019) Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors : a report from the Children ’ s Oncology Group. 6437–6448. https://​doi.​org/​10.​1002/​cam4.​2504
15.
17.
18.
go back to reference Hawkins DS. Bisogno G, Koscielniak E (2023) Introducing INSTRuCT: An international effort to promote cooperation and data sharing. Pediatr Blood Cancer 7(3):e28701 Hawkins DS. Bisogno G, Koscielniak E (2023) Introducing INSTRuCT: An international effort to promote cooperation and data sharing. Pediatr Blood Cancer 7(3):e28701
26.
go back to reference Oliveira MP, de Lima PM, A, da Silva HJ, de Mello RJV, (2014) Neoplasm seeding in biopsy tract of the musculoskeletal system. A systematic review Acta Ortop Bras 22:106–110CrossRefPubMed Oliveira MP, de Lima PM, A, da Silva HJ, de Mello RJV, (2014) Neoplasm seeding in biopsy tract of the musculoskeletal system. A systematic review Acta Ortop Bras 22:106–110CrossRefPubMed
27.
go back to reference Rogers T, Craigie R, Dall’igna P, Minard-colin V (2018) Surgery Guidelines Rhabdomyosarcoma European Paediatric Soft Tissue Sarcoma Study Group. European Pediatric Soft Tissue Sarcoma Study Group 1–18 Rogers T, Craigie R, Dall’igna P, Minard-colin V (2018) Surgery Guidelines Rhabdomyosarcoma European Paediatric Soft Tissue Sarcoma Study Group. European Pediatric Soft Tissue Sarcoma Study Group 1–18
28.
go back to reference Rogers TN, Seitz G, Fuchs J et al (2021) Surgical management of paratesticular rhabdomyosarcoma: a consensus opinion from the Children’s Oncology Group, European paediatric Soft tissue sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 68:1–10. https://doi.org/10.1002/pbc.28938CrossRef Rogers TN, Seitz G, Fuchs J et al (2021) Surgical management of paratesticular rhabdomyosarcoma: a consensus opinion from the Children’s Oncology Group, European paediatric Soft tissue sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 68:1–10. https://​doi.​org/​10.​1002/​pbc.​28938CrossRef
29.
go back to reference World Health Organization (WHO) (2020) WHO classification of tumours: soft tissue and bone tumours , 4th ed. Lyon, France World Health Organization (WHO) (2020) WHO classification of tumours: soft tissue and bone tumours , 4th ed. Lyon, France
32.
go back to reference Rudzinski ER, Kelsey A, Vokuhl C et al (2021) Pathology of childhood rhabdomyosarcoma: a consensus opinion document from the Children’s Oncology Group, European Paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 68:1–10. https://doi.org/10.1002/pbc.28798CrossRef Rudzinski ER, Kelsey A, Vokuhl C et al (2021) Pathology of childhood rhabdomyosarcoma: a consensus opinion document from the Children’s Oncology Group, European Paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 68:1–10. https://​doi.​org/​10.​1002/​pbc.​28798CrossRef
35.
go back to reference Haduong JH, Heske CM, Allen‐Rhoades W, Xue W, Teot LA, Rodeberg DA, Donaldson SS, Weiss A, Hawkins DS, Venkatramani R (2022) An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials. Pediatr Blood Cancer 69(4):e29511. https://doi.org/10.1002/pbc.29511 Haduong JH, Heske CM, Allen‐Rhoades W, Xue W, Teot LA, Rodeberg DA, Donaldson SS, Weiss A, Hawkins DS, Venkatramani R (2022) An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials. Pediatr Blood Cancer 69(4):e29511. https://​doi.​org/​10.​1002/​pbc.​29511
40.
go back to reference Morris CD, Tunn PU, Rodeberg DA, et al (2020) Surgical management of extremity rhabdomyosarcoma: a consensus opinion from the Children’s Oncology Group, the European Pediatric Soft-Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 1–8. https://doi.org/10.1002/pbc.28608 Morris CD, Tunn PU, Rodeberg DA, et al (2020) Surgical management of extremity rhabdomyosarcoma: a consensus opinion from the Children’s Oncology Group, the European Pediatric Soft-Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer 1–8. https://​doi.​org/​10.​1002/​pbc.​28608
43.
go back to reference Vaarwerk B, Bisogno G, McHugh K et al (2019) Indeterminate pulmonary nodules at diagnosis in rhabdomyosarcoma: are they clinically significant? A report from the European Paediatric Soft Tissue Sarcoma Study Group. J Clin Oncol 37:723–730. https://doi.org/10.1200/JCO.18.01535 Vaarwerk B, Bisogno G, McHugh K et al (2019) Indeterminate pulmonary nodules at diagnosis in rhabdomyosarcoma: are they clinically significant? A report from the European Paediatric Soft Tissue Sarcoma Study Group. J Clin Oncol 37:723–730. https://​doi.​org/​10.​1200/​JCO.​18.​01535
52.
go back to reference Rhee DS, Rodeberg DA, Baertschiger RM et al (2020) Update on pediatric rhabdomyosarcoma: a report from the APSA Cancer Committee. J Pediatr Surg 55:1987–1995CrossRefPubMed Rhee DS, Rodeberg DA, Baertschiger RM et al (2020) Update on pediatric rhabdomyosarcoma: a report from the APSA Cancer Committee. J Pediatr Surg 55:1987–1995CrossRefPubMed
54.
58.
go back to reference Schoot RA, Saeed P, Freling NJ, et al (2016) Local resection and brachytherapy for primary orbital rhabdomyosarcoma: outcome and failure pattern analysis. In: Ophthalmic Plastic and Reconstructive Surgery. Lippincott Williams and Wilkins, pp 354–360 Schoot RA, Saeed P, Freling NJ, et al (2016) Local resection and brachytherapy for primary orbital rhabdomyosarcoma: outcome and failure pattern analysis. In: Ophthalmic Plastic and Reconstructive Surgery. Lippincott Williams and Wilkins, pp 354–360
63.
go back to reference Defachelles AS, Bogart E, Casanova M et al (2021) Randomized phase II trial of vincristine-irinotecan with or without temozolomide, in children and adults with relapsed or refractory rhabdomyosarcoma: a European Paediatric Soft Tissue Sarcoma Study Group and Innovative Therapies for Children With Cancer. J Clin Oncol 39:2979–2990. https://doi.org/10.1200/JCO.21.00124CrossRefPubMed Defachelles AS, Bogart E, Casanova M et al (2021) Randomized phase II trial of vincristine-irinotecan with or without temozolomide, in children and adults with relapsed or refractory rhabdomyosarcoma: a European Paediatric Soft Tissue Sarcoma Study Group and Innovative Therapies for Children With Cancer. J Clin Oncol 39:2979–2990. https://​doi.​org/​10.​1200/​JCO.​21.​00124CrossRefPubMed
67.
go back to reference Oberlin O, Rey A, Sanchez De Toledo J et al (2012) Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatr. J Clin Oncol 30:2457–2465. https://doi.org/10.1200/JCO.2011.40.3287CrossRefPubMed Oberlin O, Rey A, Sanchez De Toledo J et al (2012) Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatr. J Clin Oncol 30:2457–2465. https://​doi.​org/​10.​1200/​JCO.​2011.​40.​3287CrossRefPubMed
71.
go back to reference Shah M, Denlinger CS (2015) Optimal post-treatment surveillance in cancer survivors: is more really better? Oncology (Williston Park) 230–240 Shah M, Denlinger CS (2015) Optimal post-treatment surveillance in cancer survivors: is more really better? Oncology (Williston Park) 230–240
73.
go back to reference Wille-Jørgensen P, Syk I, Smedh K et al (2018) Effect of more vs less frequent follow-up testing on overall and colorectal cancer–specific mortality in patients with stage II or III colorectal cancer the COLOFOL randomized clinical trial. JAMA - Journal of the American Medical Association 319:2095–2103. https://doi.org/10.1001/jama.2018.5623CrossRefPubMed Wille-Jørgensen P, Syk I, Smedh K et al (2018) Effect of more vs less frequent follow-up testing on overall and colorectal cancer–specific mortality in patients with stage II or III colorectal cancer the COLOFOL randomized clinical trial. JAMA - Journal of the American Medical Association 319:2095–2103. https://​doi.​org/​10.​1001/​jama.​2018.​5623CrossRefPubMed
74.
go back to reference Portman D (2018) Scanxiety. Fam Syst. Health 36:415 Portman D (2018) Scanxiety. Fam Syst. Health 36:415
77.
go back to reference Mindrup R (2022) Imprint of “Scanxiety.” AMA J Ethics 11:685–693 Mindrup R (2022) Imprint of “Scanxiety.” AMA J Ethics 11:685–693
78.
go back to reference Fetzko S, Fonseca A, Frances Wedekind M, Gupta AA, Setty BA, Schraw J, Lupo PJ, Guillerman RP, Butala AA, Russell H, Nicholls L, Walterhouse D, Hawkins DS, Okcu MF (2022) Is detection of relapse by surveillance imaging associated with longer survival in patients with rhabdomyosarcoma? Pediatr. Hematol Oncol J 44(6):305–312. https://doi.org/10.1097/MPH.0000000000002429CrossRef Fetzko S, Fonseca A, Frances Wedekind M, Gupta AA, Setty BA, Schraw J, Lupo PJ, Guillerman RP, Butala AA, Russell H, Nicholls L, Walterhouse D, Hawkins DS, Okcu MF (2022) Is detection of relapse by surveillance imaging associated with longer survival in patients with rhabdomyosarcoma? Pediatr. Hematol Oncol J 44(6):305–312. https://​doi.​org/​10.​1097/​MPH.​0000000000002429​CrossRef
79.
go back to reference Lin JL, Guillerman RP, Russell H v., et al (2013) Does routine imaging of patients for progression or relapse improve survival in rhabdomyosarcoma?Tumor. Pediatr Blood Cancer 63:202–205. https://doi.org/10.1002/pbc Lin JL, Guillerman RP, Russell H v., et al (2013) Does routine imaging of patients for progression or relapse improve survival in rhabdomyosarcoma?Tumor. Pediatr Blood Cancer 63:202–205. https://​doi.​org/​10.​1002/​pbc
81.
go back to reference World Health Orginazation (2005) Handbook for good clinical research practice (GCP): guidance for implementation. World Health Organization World Health Orginazation (2005) Handbook for good clinical research practice (GCP): guidance for implementation. World Health Organization
Metadata
Title
Imaging in rhabdomyosarcoma: a patient journey
Authors
Isabelle S. A. de Vries
Roelof van Ewijk
Laura M. E. Adriaansen
Anneloes E. Bohte
Arthur J. A. T. Braat
Raquel Dávila Fajardo
Laura S. Hiemcke-Jiwa
Marinka L. F. Hol
Simone A. J. ter Horst
Bart de Keizer
Rutger R. G. Knops
Michael T. Meister
Reineke A. Schoot
Ludi E. Smeele
Sheila Terwisscha van Scheltinga
Bas Vaarwerk
Johannes H. M. Merks
Rick R. van Rijn
Publication date
27-02-2023
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 4/2023
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-023-05596-8

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