Related Experiment Video
Updated: Dec 10, 2025
Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
Published on: September 13, 2019
Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study
Sheila E J Terwisscha van Scheltinga1, Marc H W A Wijnen1, Hélène Martelli2
1Pediatric Surgery, Pediatric Solid Tumor Unit, Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Insights
This study evaluated rhabdomyosarcoma in pediatric extremity patients, finding that improved treatment, including radiotherapy, did not enhance survival outcomes compared to prior European studies. Accurate nodal staging remains a challenge in extremity rhabdomyosarcoma management.
Area of Science:
- Pediatric Oncology
- Skeletal System Neoplasms
- Clinical Trials
Background:
- Extremity rhabdomyosarcoma presents challenges in nodal staging and local treatment.
- Previous European studies (SIOP-MMT) established benchmarks for treatment and survival.
Purpose of the Study:
- To evaluate staging, treatment, and survival outcomes in pediatric patients with localized extremity rhabdomyosarcoma within the EpSSG-RMS2005 study.
- To compare these outcomes with preceding European SIOP-MMT studies.
Main Methods:
- Analysis of 162 pediatric patients with localized extremity rhabdomyosarcoma enrolled in the EpSSG-RMS2005 study (2005-2014).
- Evaluation of histology, patient age, IRS stage, nodal status, surgical interventions, and radiotherapy delivery.
- Comparison of 5-year event-free survival (EFS) and overall survival (OS) with historical data from SIOP-MMT89 and SIOP-MMT95 studies.
Main Results:
- 113 patients (70%) had unfavorable histology, 124 (77%) were under 10 years, and 47 (29%) were node-positive.
- Regional node biopsy altered staging in 16% of cases; 96% received surgery and 73% radiotherapy.
- Five-year EFS was 58.4% and OS was 71.7%. Despite increased radiotherapy, survival did not improve compared to the SIOP-MMT95 study.
Conclusions:
- Node sampling can significantly impact lymph node staging in extremity rhabdomyosarcoma.
- Increased use of locoregional radiotherapy did not lead to improved survival in the EpSSG-RMS2005 cohort compared to the SIOP-MMT95 study.
- Challenges in nodal staging and defining optimal local treatment persist for extremity rhabdomyosarcoma.
Abstract:
Rhabdomyosarcoma of the extremities present with two main challenges: correct evaluation of initial regional nodal involvement and define adequate local treatment.
Methods:
Pediatric patients with localized rhabdomyosarcoma of the extremity included in the EpSSG-RMS2005 study between 2005 and 2014 were evaluated for staging, treatment, and survival. The outcome was compared to the preceding European SIOP-MMT studies.
Results:
Of the 162 patients included, histology was unfavorable in 113 (70%), 124 (77%) were younger than 10 years, 128 (79%) were IRS III, and 47 (29%) were node-positive. A regional node biopsy was performed in 97 patients (60%) and modified the lymph node stage in 15/97 (16%). Primary and delayed surgery was performed in 155 (96%) and radiotherapy delivered in 118 (73%) patients. Relapse occurred in 61 cases (38%), local in 14 (23%), regional in 13 (21%), distant in 22 (36%), and combined relapse in 12 (20%) with five progressive diseases (8%) and four secondary tumors (7%). Five-year event free (EFS) and overall survival (OS) were 58.4% (95%CI, 50.3-65.7) and 71.7% (63.6-78.4), respectively. In the previous studies MMT89 and MMT95, tumor surgery was performed in 32/53 (60%) and 74/82(90%), respectively, and radiotherapy delivered in 13/53 (25%) and 26/82 (30%), respectively. Five-year EFS and OS were 35.6%, and 50.3% in MMT89 and 54.3% and 68.2% in the MMT95 study.
Conclusions:
Even if the lymph node staging was not always complete according to the RMS2005 protocol, node sampling changed lymph node status in a significant number of patients. Despite the higher rate of patients treated with locoregional radiotherapy, survival in RMS2005 did not improve compared to the previous European SIOP-MMT95 study.
