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Prognostic factors of pediatric pelvic and genitourinary rhabdomyosarcoma: An analysis based on SEER database
Jiheng Wu1, Xinyi Shou2, Jiabin Cai3
1National Clinical Trial Institute, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Insights
Pediatric pelvic and genitourinary rhabdomyosarcoma (RMS) survival is influenced by factors like age and subtype, but radiation therapy (RT) showed no significant survival benefit. Further trials are needed to evaluate RT's role in treating this childhood cancer.
Area of Science:
- Pediatric Oncology
- Cancer Research
- Genitourinary Oncology
Background:
- Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children.
- Pelvic and genitourinary (GU) RMS represent a significant subset requiring prognostic evaluation.
- Understanding prognostic factors is crucial for optimizing treatment strategies in pediatric RMS.
Purpose of the Study:
- To identify prognostic factors for pediatric pelvic and GU RMS.
- To evaluate the impact of radiation therapy (RT) on survival outcomes.
- To assess survival rates in children diagnosed with pelvic and GU RMS.
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) database for patient data.
- Employed Kaplan-Meier, log-rank tests, and Cox proportional hazards models for survival analysis.
- Conducted propensity score-matched analyses to compare treatment groups, including RT vs. no RT.
Main Results:
- Embryonic RMS (79.8%) and alveolar RMS (11.1%) were the most common subtypes, with alveolar RMS having the worst prognosis.
- Testis (46.6%) was the most frequent site, followed by urinary bladder (21.8%) and prostate (18.3%).
- Favorable prognostic factors included younger age (<15 years), non-alveolar subtype, early stage, specific sites (uterus, testis), surgery, and chemotherapy. RT showed no significant difference in overall or cancer-specific survival.
Conclusions:
- A nomogram based on independent prognostic factors can predict survival rates.
- Surgery and chemotherapy are effective, but RT does not guarantee improved survival for pediatric pelvic/GU RMS.
- Prospective trials are warranted to definitively assess the role of RT in this patient population.
Background:
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcomas in children. This study aimed to investigate the prognostic factors of pelvic and genitourinary RMS in children and evaluate the survival outcomes of these children treated with or without radiation therapy (RT).
Methods:
The Surveillance, Epidemiology, and End Results Program (SEER) database was required for children with pelvic and genitourinary RMS. Overall survival (OS) and cancer-specific survival (CSS) were analyzed using the Kaplan-Meier method, log-rank test, Cox proportional hazards models, and propensity score-matched analyses.
Results:
For the 262 patients analyzed, the most common biological subtypes were embryonic (n=209, 79.8%) and alveolar (n=29, 11.1%). Patients with alveolar RMS had the worst prognosis (P < 0.05). The testis (n=122, 46.6%) was the most common location, followed by the urinary bladder (n=57, 21.8%) and prostate (n=48, 18.3%). Uterus RMS had the highest survival rate, followed by testis, urinary bladder, and prostate RMS. Favorable prognostic factors were age at diagnosis < 15 years, non-alveolar histological subtype, early tumor stage (localized/regional), specific sites (uterus and testis), and treatment (cancer-directed surgery and chemotherapy) (P < 0.05). Propensity score-matched analyses comparing the cohorts of patients treated with or without RT demonstrated no significant differences in prognostic survival (OS: P=0.872, CSS: P=0.713).
Conclusion:
The nomogram constructed based on independent prognostic factors may accurately predict survival rates at 1 and 5 years. Surgery and adjuvant chemotherapy can be effective treatments, but RT fails to guarantee a survival benefit. Therefore, prospective trials evaluating RT for pediatric pelvic and genitourinary RMS are warranted.
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