The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data

Gianni Bisogno; Monika Sparber-Sauer; David Rodeberg; Johannes H.M. Merks; Ewa Koscielniak; Suzanne L. Wolden; Gian Luca De Salvo; Paola Del Bianco; Wei Xue; Martin Ebinger; Christian Vokuhl; Rajkumar Venkatramani; Samuel L. Volchenboum; Brian Furner; Veronique Minard Colin; Douglas S. Hawkins

doi:10.1002/cncr.35974

The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data

Gianni Bisogno, Monika Sparber-Sauer, David Rodeberg, Johannes H.M. Merks, Ewa Koscielniak, Suzanne L. Wolden, Gian Luca De Salvo, Paola Del Bianco, Wei Xue, Martin Ebinger, Christian Vokuhl, Rajkumar Venkatramani, Samuel L. Volchenboum, Brian Furner, Veronique Minard Colin, Douglas S. Hawkins

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Background: The International Soft Tissue Sarcoma Consortium (INSTRuCT) was established in 2017 to enhance international collaboration. This study describes the characteristics of rhabdomyosarcoma (RMS) patients in the INSTRuCT database and examines differences across contributing groups. Methods: INSTRuCT includes data Children’s Oncology Group (COG), Cooperative Weichteilsarkom Studiengruppe (CWS), and European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) along with prior studies from Malignant Mesenchymal Tumour Committee (MMT) and Italian Soft Tissue Sarcoma Committee (STSC). Data standardization was supported by the University of Chicago’s Pediatric Cancer Data Commons. Pseudonymized patient-level data from clinical trials were harmonized. Differences across groups were assessed using χ² or Kruskal–Wallis tests. Results: As of March 2025, INSTRuCT includes 6972 RMS patients from 16 trials (1990–2016). Embryonal RMS was the most common histology in all groups (range, 45.4%–62.2%). Alveolar RMS was less frequent in EpSSG (26.8%) although the rate of RMS fusion-positive was comparable across groups (74.6%–81.9%). COG and EpSSG had more T1 tumors, (53.2% and 51.4%) with COG reporting more tumors <5 cm (52%). Nodal involvement was least reported in MMT (15.4%). Metastatic patients were less represented in MMT (11%) and EpSSG (13.3%). Tumor site distribution varied: genitourinary nonbladder/prostate RMS was more common in COG, whereas head and neck nonparameningeal and orbital RMS were more represented in MMT and STSC. MMT had fewer completely resected tumors (8.9%). Conclusion: Differences among RMS study populations reflect evolving diagnostic criteria and treatment strategies that should be considered in future analyses. INSTRuCT offers a valuable international data set for RMS research.

Original language	English
Article number	e35974
Journal	Cancer
Volume	131
Issue number	14
DOIs	https://doi.org/10.1002/cncr.35974
State	Published - Jul 15 2025

Bibliographical note

Publisher Copyright:
© 2025 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.

Funding

The International Soft Tissue Sarcoma Consortium and the Pediatric Cancer Data Commons are supported in part by Cancer Research Foundation, Children’s Research Foundation, Comer Development Board, KickCancer, King Baudouin Foundation, Rally Foundation for Childhood Cancer Research, Seattle Children’s Foundation from Kat’s Crew Guild through the Sarcoma Research Fund, St. Baldrick’s Foundation, and The Andrew McDonough B+ Foundation. This work is made possible through the efforts of Children’s Oncology Group, Cooperative Weichteilsarkom Studiengruppe der GPOH, The European Paediatric Soft Tissue Sarcoma Study Group, MMT Malignant Mesenchymal Tumour Committee, and STSC AIEOP Italian Soft Tissue Sarcoma Committee. We thank Suzi Birz, INSTRuCT consortium manager, for her exceptional support and dedication in managing all INSTRuCT scientific and administrative tasks. No direct funds have been received for this study. All patient data were collected under applicable ethical approval based on the country of clinical trial enrollment with patient or parent consent as determined by national ethical approval requirements. Open access publishing facilitated by Universita degli Studi di Padova, as part of the Wiley - CRUI-CARE agreement. The International Soft Tissue Sarcoma Consortium and the Pediatric Cancer Data Commons are supported in part by Cancer Research Foundation, Children’s Research Foundation, Comer Development Board, KickCancer, King Baudouin Foundation, Rally Foundation for Childhood Cancer Research, Seattle Children’s Foundation from Kat’s Crew Guild through the Sarcoma Research Fund, St. Baldrick’s Foundation, and The Andrew McDonough B+ Foundation. This work is made possible through the efforts of Children’s Oncology Group, Cooperative Weichteilsarkom Studiengruppe der GPOH, The European Paediatric Soft Tissue Sarcoma Study Group, MMT Malignant Mesenchymal Tumour Committee, and STSC AIEOP Italian Soft Tissue Sarcoma Committee. We thank Suzi Birz, INSTRuCT consortium manager, for her exceptional support and dedication in managing all INSTRuCT scientific and administrative tasks. No direct funds have been received for this study. All patient data were collected under applicable ethical approval based on the country of clinical trial enrollment with patient or parent consent as determined by national ethical approval requirements. Open access publishing facilitated by Universita degli Studi di Padova, as part of the Wiley ‐ CRUI‐CARE agreement. Brian Furner reports stock holdings with Cisco Systems, Microsoft Corporation, and United Therapeutics Corporation. Johannes H. M. Merks reports consulting fees from Bayer, GlaxoSmithKline, and Merck. Monika Sparber‐Sauer reports grant and/or contract funding from Deutsche Kinderkrebsstiftung and Deutsche Krebshilfe. Samuel L. Volchenboum reports consulting fees from Belay Diagnostics and CVS Accordant; and stock holdings with Litmus Health. The other authors declare no conflicts of interest.

Funders	Funder number
Children’s Oncology Group
MMT Malignant Mesenchymal Tumour Committee
International Soft Tissue Sarcoma Consortium
Andrew McDonough B+ Foundation
Cancer Research Foundation
Koning Boudewijnstichting
STSC AIEOP Italian Soft Tissue Sarcoma Committee
Cincinnati Children’s Research Foundation
Università degli Studi di Padova
Deutsche Krebshilfe
St. Baldrick's Foundation
Comer Development Board
European Paediatric Soft Tissue Sarcoma Study Group
Deutsche Kinderkrebsstiftung
Rally Foundation
Cooperative Weichteilsarkom Studiengruppe der GPOH

Keywords

INSTRuCT
International Soft Tissue Sarcoma Consortium
Pediatric Cancer Data Commons
rhabdomyosarcoma

ASJC Scopus subject areas

Oncology
Cancer Research

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1002/cncr.35974

Cite this

Bisogno, G., Sparber-Sauer, M., Rodeberg, D., Merks, J. H. M., Koscielniak, E., Wolden, S. L., De Salvo, G. L., Del Bianco, P., Xue, W., Ebinger, M., Vokuhl, C., Venkatramani, R., Volchenboum, S. L., Furner, B., Minard Colin, V., & Hawkins, D. S. (2025). The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data. Cancer, 131(14), Article e35974. https://doi.org/10.1002/cncr.35974

@article{42472d15df3e499c86b5a0da37c25a27,

title = "The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data",

abstract = "Background: The International Soft Tissue Sarcoma Consortium (INSTRuCT) was established in 2017 to enhance international collaboration. This study describes the characteristics of rhabdomyosarcoma (RMS) patients in the INSTRuCT database and examines differences across contributing groups. Methods: INSTRuCT includes data Children{\textquoteright}s Oncology Group (COG), Cooperative Weichteilsarkom Studiengruppe (CWS), and European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) along with prior studies from Malignant Mesenchymal Tumour Committee (MMT) and Italian Soft Tissue Sarcoma Committee (STSC). Data standardization was supported by the University of Chicago{\textquoteright}s Pediatric Cancer Data Commons. Pseudonymized patient-level data from clinical trials were harmonized. Differences across groups were assessed using χ2 or Kruskal–Wallis tests. Results: As of March 2025, INSTRuCT includes 6972 RMS patients from 16 trials (1990–2016). Embryonal RMS was the most common histology in all groups (range, 45.4\%–62.2\%). Alveolar RMS was less frequent in EpSSG (26.8\%) although the rate of RMS fusion-positive was comparable across groups (74.6\%–81.9\%). COG and EpSSG had more T1 tumors, (53.2\% and 51.4\%) with COG reporting more tumors <5 cm (52\%). Nodal involvement was least reported in MMT (15.4\%). Metastatic patients were less represented in MMT (11\%) and EpSSG (13.3\%). Tumor site distribution varied: genitourinary nonbladder/prostate RMS was more common in COG, whereas head and neck nonparameningeal and orbital RMS were more represented in MMT and STSC. MMT had fewer completely resected tumors (8.9\%). Conclusion: Differences among RMS study populations reflect evolving diagnostic criteria and treatment strategies that should be considered in future analyses. INSTRuCT offers a valuable international data set for RMS research.",

keywords = "INSTRuCT, International Soft Tissue Sarcoma Consortium, Pediatric Cancer Data Commons, rhabdomyosarcoma",

author = "Gianni Bisogno and Monika Sparber-Sauer and David Rodeberg and Merks, \{Johannes H.M.\} and Ewa Koscielniak and Wolden, \{Suzanne L.\} and \{De Salvo\}, \{Gian Luca\} and \{Del Bianco\}, Paola and Wei Xue and Martin Ebinger and Christian Vokuhl and Rajkumar Venkatramani and Volchenboum, \{Samuel L.\} and Brian Furner and \{Minard Colin\}, Veronique and Hawkins, \{Douglas S.\}",

note = "Publisher Copyright: {\textcopyright} 2025 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.",

year = "2025",

month = jul,

day = "15",

doi = "10.1002/cncr.35974",

language = "English",

volume = "131",

number = "14",

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Bisogno, G, Sparber-Sauer, M, Rodeberg, D, Merks, JHM, Koscielniak, E, Wolden, SL, De Salvo, GL, Del Bianco, P, Xue, W, Ebinger, M, Vokuhl, C, Venkatramani, R, Volchenboum, SL, Furner, B, Minard Colin, V & Hawkins, DS 2025, 'The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data', Cancer, vol. 131, no. 14, e35974. https://doi.org/10.1002/cncr.35974

TY - JOUR

T1 - The International Soft Tissue Sarcoma Consortium

T2 - The baseline analysis of rhabdomyosarcoma data

AU - Bisogno, Gianni

AU - Sparber-Sauer, Monika

AU - Rodeberg, David

AU - Merks, Johannes H.M.

AU - Koscielniak, Ewa

AU - Wolden, Suzanne L.

AU - De Salvo, Gian Luca

AU - Del Bianco, Paola

AU - Xue, Wei

AU - Ebinger, Martin

AU - Vokuhl, Christian

AU - Venkatramani, Rajkumar

AU - Volchenboum, Samuel L.

AU - Furner, Brian

AU - Minard Colin, Veronique

AU - Hawkins, Douglas S.

PY - 2025/7/15

Y1 - 2025/7/15

N2 - Background: The International Soft Tissue Sarcoma Consortium (INSTRuCT) was established in 2017 to enhance international collaboration. This study describes the characteristics of rhabdomyosarcoma (RMS) patients in the INSTRuCT database and examines differences across contributing groups. Methods: INSTRuCT includes data Children’s Oncology Group (COG), Cooperative Weichteilsarkom Studiengruppe (CWS), and European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) along with prior studies from Malignant Mesenchymal Tumour Committee (MMT) and Italian Soft Tissue Sarcoma Committee (STSC). Data standardization was supported by the University of Chicago’s Pediatric Cancer Data Commons. Pseudonymized patient-level data from clinical trials were harmonized. Differences across groups were assessed using χ2 or Kruskal–Wallis tests. Results: As of March 2025, INSTRuCT includes 6972 RMS patients from 16 trials (1990–2016). Embryonal RMS was the most common histology in all groups (range, 45.4%–62.2%). Alveolar RMS was less frequent in EpSSG (26.8%) although the rate of RMS fusion-positive was comparable across groups (74.6%–81.9%). COG and EpSSG had more T1 tumors, (53.2% and 51.4%) with COG reporting more tumors <5 cm (52%). Nodal involvement was least reported in MMT (15.4%). Metastatic patients were less represented in MMT (11%) and EpSSG (13.3%). Tumor site distribution varied: genitourinary nonbladder/prostate RMS was more common in COG, whereas head and neck nonparameningeal and orbital RMS were more represented in MMT and STSC. MMT had fewer completely resected tumors (8.9%). Conclusion: Differences among RMS study populations reflect evolving diagnostic criteria and treatment strategies that should be considered in future analyses. INSTRuCT offers a valuable international data set for RMS research.

AB - Background: The International Soft Tissue Sarcoma Consortium (INSTRuCT) was established in 2017 to enhance international collaboration. This study describes the characteristics of rhabdomyosarcoma (RMS) patients in the INSTRuCT database and examines differences across contributing groups. Methods: INSTRuCT includes data Children’s Oncology Group (COG), Cooperative Weichteilsarkom Studiengruppe (CWS), and European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) along with prior studies from Malignant Mesenchymal Tumour Committee (MMT) and Italian Soft Tissue Sarcoma Committee (STSC). Data standardization was supported by the University of Chicago’s Pediatric Cancer Data Commons. Pseudonymized patient-level data from clinical trials were harmonized. Differences across groups were assessed using χ2 or Kruskal–Wallis tests. Results: As of March 2025, INSTRuCT includes 6972 RMS patients from 16 trials (1990–2016). Embryonal RMS was the most common histology in all groups (range, 45.4%–62.2%). Alveolar RMS was less frequent in EpSSG (26.8%) although the rate of RMS fusion-positive was comparable across groups (74.6%–81.9%). COG and EpSSG had more T1 tumors, (53.2% and 51.4%) with COG reporting more tumors <5 cm (52%). Nodal involvement was least reported in MMT (15.4%). Metastatic patients were less represented in MMT (11%) and EpSSG (13.3%). Tumor site distribution varied: genitourinary nonbladder/prostate RMS was more common in COG, whereas head and neck nonparameningeal and orbital RMS were more represented in MMT and STSC. MMT had fewer completely resected tumors (8.9%). Conclusion: Differences among RMS study populations reflect evolving diagnostic criteria and treatment strategies that should be considered in future analyses. INSTRuCT offers a valuable international data set for RMS research.

KW - INSTRuCT

KW - International Soft Tissue Sarcoma Consortium

KW - Pediatric Cancer Data Commons

KW - rhabdomyosarcoma

UR - https://www.scopus.com/pages/publications/105010151756

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U2 - 10.1002/cncr.35974

DO - 10.1002/cncr.35974

M3 - Article

C2 - 40632016

AN - SCOPUS:105010151756

SN - 0008-543X

VL - 131

JO - Cancer

JF - Cancer

IS - 14

M1 - e35974

ER -

The International Soft Tissue Sarcoma Consortium: The baseline analysis of rhabdomyosarcoma data

Abstract

Bibliographical note

Funding

Keywords

ASJC Scopus subject areas

UN SDGs

Access to Document

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