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Bilateral Wilms' tumors: a single-center experience with 19 cases
Alastair J W Millar1, Alan Davidson, Heinz Rode
1Department of Paediatric Surgery, Red Cross Children's Hospital, School of Child and Adolescent Health, University of Cape Town, Cape Town, South Africa.
Journal of Pediatric Surgery
|August 6, 2005
Summary
Bilateral Wilms tumors require careful management to eradicate cancer while preserving kidney function. Innovative surgical techniques and chemotherapy offer good outcomes, especially when nephroblastomatosis is monitored closely.
Area of Science:
- Pediatric Oncology
- Nephrology
- Surgical Oncology
Background:
- Bilateral Wilms tumors pose significant therapeutic challenges.
- Management is complicated by delayed presentation, poor chemotherapy response, and nephroblastomatosis.
- The primary goals are complete tumor eradication and renal function preservation.
Purpose of the Study:
- To evaluate the outcomes of treating bilateral Wilms tumors.
- To assess the efficacy of neoadjuvant chemotherapy and conservative surgical approaches.
- To analyze the impact of nephroblastomatosis and metastatic spread on patient survival.
Main Methods:
- Retrospective review of 19 bilateral Wilms tumor cases (1981-2003).
- Treatment followed National Wilms Tumor Study Group protocols, including bilateral biopsy, neoadjuvant chemotherapy, and tumorectomy.
- Surgical interventions included nephrectomy with renal reconstruction and revision tumorectomy for recurrence.
Main Results:
- Ten patients achieved long-term disease-free survival with preserved renal function.
- Six of seven patients with extrarenal spread died, indicating a poor prognosis.
- All patients with unfavorable histology survived, and 4 of 5 with metachronous presentations are alive.
Conclusions:
- Nephron-sparing surgery and chemotherapy yield good results with preserved renal function.
- Nephroblastomatosis is common and necessitates vigilant monitoring for tumor recurrence.
- Metastatic spread outside the kidney is associated with a poor prognosis, while unfavorable histology did not impact survival in this series.