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Patterns of Failure in Parameningeal Alveolar Rhabdomyosarcoma.
Julie A Bradley1, Daniel J Indelicato1, Haruka Uezono1
1Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida.
International Journal of Radiation Oncology, Biology, Physics
|February 12, 2020
Summary
Pediatric parameningeal alveolar rhabdomyosarcoma patients treated with proton radiation therapy showed a 58% overall survival rate. Intracranial extension and N1 disease negatively impacted survival, suggesting treatment modifications are needed.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Medical Physics
Background:
- Parameningeal alveolar rhabdomyosarcoma (PARMS) is a rare but aggressive pediatric cancer.
- Treatment strategies for PARMS, particularly involving radiation therapy, require ongoing evaluation to improve outcomes.
Purpose of the Study:
- To analyze failure patterns, clinical outcomes, and prognostic factors in pediatric patients with PARMS treated with radiation therapy.
- To identify factors influencing disease control and survival in this patient cohort.
Main Methods:
- Retrospective review of 24 pediatric patients (≤21 years) with PARMS treated between 2009-2016.
- Analysis of clinical and treatment planning data, including concurrent chemotherapy and proton radiation therapy.
- Kaplan-Meier and log-rank tests used to assess disease control, survival rates, and prognostic factors.
Main Results:
- The 3-year local control, regional control, disease-free survival, and overall survival rates were 66%, 94%, 40%, and 58%, respectively.
- N1 disease and intracranial extension (ICE) were associated with inferior overall survival.
- Female sex correlated with better local control; younger age (<4 years) and absence of ICE improved freedom from distant metastases.
Conclusions:
- While regional control was high, local recurrences and leptomeningeal progression in patients with ICE highlight the need for refined local and central nervous system radiation strategies.
- Prognostic factors such as N1 disease, ICE, and age significantly influence outcomes in pediatric PARMS, guiding future treatment decisions.
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