From the Guidelines
Rhabdomyosarcoma is the most common sarcoma in children, accounting for approximately 15% of pediatric malignancies. This soft tissue malignancy originates from primitive muscle cells and typically presents as a painless mass, with the most common locations being the head and neck region, genitourinary tract, and extremities 1. There are two main histological subtypes: embryonal (more common and generally has a better prognosis) and alveolar (more aggressive). According to the most recent guidelines, treatment of rhabdomyosarcoma is commonly guided by International Clinical Trial protocols such as FaR-RMS 1.
Key Characteristics of Rhabdomyosarcoma
- Accounts for approximately 3-4% of all pediatric cancers
- Typically presents as a painless mass
- Most common locations: head and neck region, genitourinary tract, and extremities
- Two main histological subtypes: embryonal and alveolar
Treatment Approach
Treatment typically involves a multimodal approach including surgery, chemotherapy, and radiation therapy. The specific treatment regimen depends on the tumor's location, size, stage, and histological subtype. Early diagnosis is crucial for improved outcomes, with overall survival rates ranging from 70-80% for localized disease to around 30% for metastatic disease. Children with rhabdomyosarcoma should be treated at specialized pediatric cancer centers where multidisciplinary teams can provide comprehensive care 1.
Management Considerations
- Close working between children’s cancer MDTs and sarcoma MDTs is regarded as best practice for managing non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) in children and young people
- Management of NRSTS is likely to be similar in all age groups, with similar application of chemotherapy and radiotherapy 1
From the Research
Definition and Prevalence of Rhabdomyosarcoma
- Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, representing a high-grade neoplasm of skeletal myoblast-like cells 2, 3, 4, 5.
- It accounts for 3% of all pediatric tumors and is the third most common extracranial malignant solid tumor in children and adolescents 6, 5.
Subtypes and Characteristics of Rhabdomyosarcoma
- The two major subtypes of RMS are embryonal (ERMS) and alveolar (ARMS), which are distinct in their morphology and genetic make-up 2, 3.
- Other subtypes include pleomorphic, spindle cell, mixed-type, and rhabdomyosarcoma with ganglionic differentiation 5.
- The prognosis for RMS depends strongly on tumor size, location, staging, and child's age, with ERMS having a more favorable outcome than ARMS 3.
Treatment and Survival Rates of Rhabdomyosarcoma
- Multimodality therapy, including surgery, chemotherapy, and radiation therapy, is the mainstay of treatment for RMS 2, 6.
- Improvements in risk stratification and treatment have led to increased 5-year survival rates, from 25% to 70% over the past 40 years 6.
- The 5-year survival rate varies by subtype, with embryonal RMS having the highest rate (73.9%) and pleomorphic and alveolar RMS having the worst overall survival 5.