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Update on pediatric rhabdomyosarcoma: A report from the APSA Cancer Committee
Daniel S Rhee1, David A Rodeberg2, Reto M Baertschiger3
1Division of Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD.
Insights
Pediatric rhabdomyosarcoma management is evolving. FOXO1 fusion status aids risk stratification, and while surgical lymph node assessment is crucial, hemiscrotectomy is not recommended.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma.
- Multimodality treatment is essential for managing this condition.
Purpose of the Study:
- To provide an updated review of risk stratification strategies.
- To summarize current surgical and medical management approaches for pediatric rhabdomyosarcoma.
Main Methods:
- Comprehensive literature review of pediatric rhabdomyosarcoma.
- Inclusion of recent Children's Oncology Group and international collaborative studies.
Main Results:
- FOXO1 fusion status is a superior prognostic factor for risk stratification compared to histology.
- FDG-PET-CT has limited accuracy for detecting nodal metastasis; surgical assessment is required for specific sites and ages.
- Hemiscrotectomy for paratesticular rhabdomyosarcoma does not improve survival and is not advised.
Conclusions:
- Treatment strategies for pediatric rhabdomyosarcoma are continuously advancing.
- This review offers evidence-based standards for treatment, focusing on surgical interventions.
Background/Purpose:
Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma.
Methods:
A comprehensive review of the current literature on pediatric rhabdomyosarcoma, including the most recent Children's Oncology Group studies and several international collaboratives, was performed by the authors and key findings were summarized in the manuscript.
Results:
FOXO1 fusion status is a stronger prognostic factor than histology and is now used for risk stratification in treatment protocols. For assessment of regional nodal involvement, FDG-PET-CT shows poor sensitivity and specificity to detect histologically confirmed nodal metastasis. Thus, surgical assessment of regional lymph nodes is required for rhabdomyosarcoma of the extremities or trunk as well as paratesticular rhabdomyosarcoma in patients ≥10 years of age, although adherence to surgical guidelines remains poor. Hemiscrotectomy performed for scrotal violation in paratesticular rhabdomyosarcoma has not shown an improvement in event free survival and is not recommended.
Conclusions:
Surgical and medical treatment strategies for rhabdomyosarcoma in children continue to evolve. This review provides current evidence-based treatment standards with an emphasis on surgical care.
Type Of Study:
Review.
Level Of Evidence:
Level IV.
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