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Updated: Jun 22, 2026

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
Published on: September 13, 2019
[Effect of protocol RS-99 for childhood rhabdomyosarcoma]
Jing-Yan Tang1, Ci Pan, Min Xu
1Department of Hemotology/ Oncology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University,Shanghai 200127, China.
Insights
The RS-99 protocol shows promise for treating childhood rhabdomyosarcoma (RS) in early stages, but is less effective for advanced stage IV disease. Further research is needed for stage IV rhabdomyosarcoma treatment.
Area of Science:
- Pediatric Oncology
- Cancer Therapeutics
- Clinical Protocol Development
Context:
- Childhood rhabdomyosarcoma (RS) presents a significant challenge in pediatric oncology.
- Existing treatment protocols require optimization to improve patient outcomes.
- Staging and risk stratification are crucial for tailoring rhabdomyosarcoma treatment.
Purpose:
- To evaluate the efficacy of the novel RS-99 protocol for childhood rhabdomyosarcoma.
- To establish a treatment strategy that improves the prognosis of rhabdomyosarcoma.
- To assess the protocol's effectiveness across different stages of rhabdomyosarcoma.
Summary:
- The RS-99 protocol, involving chemotherapy regimens (VCP, IeV, AVCP, IEV, DEV) and radiotherapy, was applied to 30 pediatric rhabdomyosarcoma patients.
- Complete response (CR) was achieved in 23 patients, with 17 maintaining CR for a median of 37 months.
- Event-free survival (EFS) was 47% and overall survival (OS) was 52% at 5 years, with notable success in stages I-III but limited efficacy in stage IV.
Impact:
- The RS-99 protocol demonstrates significant effectiveness for stages I, II, and III rhabdomyosarcoma.
- The protocol's limited impact on stage IV rhabdomyosarcoma highlights a critical unmet need.
- Findings provide a basis for refining rhabdomyosarcoma treatment strategies and future clinical trials.
Objective:
To develop a reasonable protocol for childhood rhabdomyosarcoma (RS) to improve the prognosis of this disease.
Methods:
The protocol RS-99, developed on the base of pathological examination, clinical staging and grouping was used for 30 RS patients, 15 male and 15 female, aged 53 months (15-180 months). For the low-risk patients the regimen VCP and IeV were used alternately for 6 treatment courses and then local radiotherapy was given, for the median and high risk patients, regimen AVCP and IEV were used alternately for 6 courses, local radiotherapy and selective operation were performed, and then DEV and IeV were used alternately for 6 courses, and for the high-risk patients DEV and IeV were used alternately for 18 courses in total and then hematopoietic stem cell transplantation was conducted.
Results:
Twenty-three of the 30 patients reached complete response (CR) 17 of which remained in CR for 37 (32-92) months, and 7 of the 30 patients achieved partial response (PR) but their condition worsened later. Relapse was seen in 6 patients 15 (7-38) months later. The 5 year event-free survival (EFS) rate was 47% and the overall survival (OS) rate was 52%. All the 10 stage I and II patients, 5 of the 8 stage III patients, and only 2 of the 12 stage IV patients still remained in CR.
Conclusion:
The protocol RS-99 is effective on the RS at stages I, II, and III, however, is ineffective on the disease at stage IV.
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