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Genitourinary rhabdomyosarcoma: lessons from a developing-world series
Richard Wood1, John Lazarus, Alan Davidson
1Red Cross Children's Hospital, Paediatric Surgery, Rondebosch 7701, South Africa.
Insights
Pediatric genitourinary rhabdomyosarcoma in developing nations presents with more advanced disease. Improved surgical and oncological care since 1992 has significantly boosted survival rates for these children.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Global Health
Background:
- Genitourinary rhabdomyosarcoma (GU-RMS) is a rare pediatric malignancy.
- Data from developing countries, particularly Africa, is limited.
- Understanding disease presentation and outcomes is crucial for improving care.
Purpose of the Study:
- To retrospectively review a large cohort of pediatric patients with GU-RMS from a developing country.
- To analyze clinical presentation, demographics, and oncological management.
- To compare outcomes before and after 1992.
Main Methods:
- Retrospective review of 49 pediatric patients treated between 1961 and 2008.
- Analysis included clinical presentation, demographics, surgical records, histology, and management.
- Patients were grouped into pre-1992 and post-1992 treatment cohorts.
Main Results:
- Median age at presentation was 3.5 years.
- Most patients (59%) had locally advanced disease (Intergroup Rhabdomyosarcoma Study group 3).
- Tumors >10 cm and positive lymph nodes were common (41% and 33%, respectively).
- Overall survival was 65% (30/46).
- Survival improved significantly for patients treated post-1992 (80% vs. 56%, P=.04).
Conclusions:
- Pediatric GU-RMS in this African series presents with more advanced, bulky, and node-positive disease compared to developed nations.
- Improvements in surgical and oncological care over the past two decades have enhanced survival for children with locally advanced GU-RMS.
Objective:
The objective was to retrospectively review a large series of pediatric patients with genitourinary rhabdomyosarcoma from a developing country.
Methods:
A total of 49 children were treated over a 47-year period (1961-2008). Analysis of the clinical presentation, demographics, surgical records, histological results, and oncological management was performed. The patients were analyzed as a whole and also in 2 separate groups (pre- and post-1992).
Results:
The median age at clinical presentation was 3½ years. The majority (59%) of patients were Intergroup Rhabdomyosarcoma Study group 3, with locally advanced disease at presentation. Twenty (41%) of the 49 patients presented with primary tumors greater than 10 cm in diameter. Sixteen (33%) of the 49 patients had positive regional lymph nodes at presentation. The overall survival of the series was 30 (65%) of 46. The survival for those treated after 1992 in Intergroup Rhabdomyosarcoma Study group 3 was superior (P = .04) to those treated before 1992 (80% vs 56%).
Conclusion:
Children in this large African series of genitourinary rhabdomyosarcoma present with greater locally advanced disease (node positive and bulky disease) when compared with the developed world. Improvements in the last 2 decades in local surgical and oncological care have led to an improvement in survival in children with locally advanced disease.
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