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Bladder management in children with genitourinary sarcoma
B A Hicks1, T W Hensle, K A Burbige
1Division of Pediatric Surgery, Babies Hospital, Columbia-Presbyterian Medical Center, New York, NY 10032.
Journal of Pediatric Surgery
|August 1, 1993
Summary
Pediatric genitourinary sarcoma treatment evolved towards less radical surgery. Partial cystectomy with reconstruction offers functional bladders, while chemotherapy and radiotherapy alone risk bladder deterioration.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Urologic Oncology
Background:
- Genitourinary sarcoma is a rare pediatric malignancy.
- Treatment strategies have evolved over time.
- Rhabdomyosarcoma is the most common histological subtype.
Purpose of the Study:
- To evaluate treatment outcomes for pediatric genitourinary sarcoma.
- To assess the impact of surgical intervention and radiotherapy on bladder function.
- To identify optimal treatment approaches for improved functional outcomes.
Main Methods:
- Retrospective review of 14 patients treated between 1977 and 1991.
- Analysis of primary tumor site, histology, and treatment modalities (surgery, chemotherapy, radiotherapy).
- Assessment of survival rates, surgical margins, and bladder function post-treatment.
Main Results:
- 100% overall survival with follow-up ranging from 6 to 168 months.
- Partial cystectomy with reconstruction resulted in negative margins, no tumor recurrence, and volitional voiding.
- 50% of patients treated with chemotherapy and radiotherapy alone experienced significant bladder deterioration requiring reconstruction.
Conclusions:
- Surgical resection remains the primary curative modality for pediatric genitourinary sarcoma.
- Partial cystectomy, with or without primary reconstruction, is a preferable alternative to exenteration for selected patients.
- Long-term bladder function requires careful monitoring in patients treated with chemotherapy and radiotherapy alone.