Factors associated with relapse/progression in pediatric trunk and extremity rhabdomyosarcoma - Report - MDSpire

Factors associated with relapse/progression in pediatric trunk and extremity rhabdomyosarcoma

  • By

  • Yanhua Li

  • Huanhuan Zhang

  • Jingbo Shao

  • Xuelian Liao

  • Yangyang Jiao

  • Shayi Jiang

  • Jingwei Yang

  • July 2, 2026

  • 0 min

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Clinical Characteristics and Risk Factors Linked to Relapse in Pediatric RMS

Overview

This study investigates the clinical characteristics and risk factors associated with relapse and progression in pediatric patients with rhabdomyosarcoma (RMS) of the trunk and extremities. Key findings indicate a 40% relapse/progression rate, with significant associations found for metastasis at diagnosis and macroscopic residual disease post-surgery.

Background

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children, representing a significant proportion of pediatric malignancies. RMS of the trunk and extremities is particularly concerning due to its association with higher rates of metastasis and relapse, leading to unfavorable outcomes.

Data Highlights

OutcomeRate95% CI
5-year event-free survival60%34.5%-85.5%
5-year overall survival66.7%42.1%-91.3%
Overall relapse/progression rate40%16.8%-68.7%

Key Findings

  • The median follow-up duration was 48 months.
  • Relapse/progression rates were significantly higher in patients with metastasis at diagnosis (85.7% vs 0%, P<0.001).
  • High-risk stratification was associated with a 75.0% relapse/progression rate (P=0.010).
  • Patients with macroscopic residual disease after surgery had a 100% relapse/progression rate (P<0.001).
  • Regional lymph node involvement showed a trend toward higher relapse/progression rates (66.7% vs 22.2%, P=0.123).

Clinical Implications

Clinicians should consider the identified risk factors when monitoring patients for relapse.

Conclusion

Pediatric trunk and extremity RMS presents a high risk of relapse and progression, with metastasis at diagnosis and residual disease post-surgery identified as critical prognostic factors.

Related Resources & Content

  1. Gianni Bisogno, MD, PhD, Journal of Clinical Oncology, 2025 -- High-Risk Rhabdomyosarcoma: Long-Term Outcomes With Maintenance Chemotherapy
  2. Blood Cancer Journal -- Identification of a genetically defined ultra-high-risk group in relapsed pediatric T-lymphoblastic leukemia
  3. The ASCO Post -- Outcomes and Patterns of Relapse in Pediatric Medulloblastoma
  4. Childhood Rhabdomyosarcoma Treatment (PDQ®) - NCI
  5. European standard clinical practice recommendations for children and adolescents with Rhabdomyosarcoma, ScienceDirect
  6. NCCN Publishes New Guidelines Focusing on Pediatric Rhabdomyosarcoma | CancerNetwork
  7. Frontiers in Pediatrics — Current treatment landscape and translational priorities in malignant rhabdoid tumor of the kidney
  8. Childhood Rhabdomyosarcoma Treatment (PDQ®) - NCI
  9. European standard clinical practice recommendations for children and adolescents with Rhabdomyosarcoma a joint EpSSG, CWS and ERN PaedCan project - ScienceDirect
  10. NCCN Publishes New Guidelines Focusing on Pediatric Rhabdomyosarcoma | CancerNetwork
  11. Previously Untreated Intermediate-Risk Rhabdomyosarcoma Addition of Temsirolimus to Chemotherapy in Young Patients - The ASCO Post
  12. Surgical Lymph Node Staging in Extremity Rhabdomyosarcoma: The EpSSG RMS 2005 Trial Experience | Annals of Surgical Oncology | Springer Nature Link
  13. Relapsed rhabdomyosarcoma: treatment recommendations from the European pediatric soft tissue sarcoma study group (EpSSG) | British Journal of Cancer

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