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Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study
Reineke A Schoot1, Pieter Taselaar1, Giovanni Scarzello2
1Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Background:
Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.
Methods:
Patients with PM RMS were treated within the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study with risk-adapted, multi-modal treatment.
Results:
Three-hundred-eighty-one patients with PM RMS were included. Radiotherapy was administered in 359 patients (77 with surgery). After a median follow-up of 75 months, 5-year event-free survival was 60% (95% confidence interval (CI) 55%-65%), 5-year overall survival was 65% (95% CI 60%-70%).
Conclusions:
The outcome for patients with PM RMS has not improved in comparison to previous historical studies, despite the more rigorous application of radiotherapy (94% of patients). Signs of meningeal involvement, PM site, and age at diagnosis remained prognostic risk factors.
Trial Registration:
EudraCT number 2005-000217-35.
Insights
Treatment for parameningeal rhabdomyosarcoma (RMS) in children did not improve survival outcomes. Meningeal involvement, tumor site, and age remain key prognostic factors for pediatric rhabdomyosarcoma.
Area of Science:
- Pediatric Oncology
- Cancer Research
- Sarcoma Treatment
Background:
- Parameningeal (PM) rhabdomyosarcoma (RMS) is associated with poor prognosis.
- Previous studies indicate unfavorable outcomes for PM RMS.
- Risk stratification is crucial for managing PM RMS.
Purpose of the Study:
- To describe treatment and outcomes for pediatric patients with PM RMS.
- To investigate the prognostic value of risk factors in PM RMS.
- To evaluate the effectiveness of risk-adapted, multi-modal therapy in PM RMS.
Main Methods:
- Patients with PM RMS were enrolled in the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study.
- Treatment involved risk-adapted, multi-modal therapeutic approaches.
- PM site was scored by originating site and highest risk extension.
Main Results:
- 381 patients with PM RMS were included in the study.
- Radiotherapy was administered to 359 patients (77 with surgery).
- Five-year event-free survival was 60%, and overall survival was 65%.
Conclusions:
- Outcomes for PM RMS patients have not improved compared to historical data.
- Rigorous radiotherapy application (94%) did not enhance survival.
- Meningeal involvement, PM site, and age at diagnosis are significant prognostic factors.
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