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Management of Wilms' tumor with intracardiac extension
S T Schettini1, J H da Fonseca, S C Abib
1Department of Pediatric Surgery, Federal University of São Paulo, Escola Paulista de Medicina, Brazil.
Insights
Wilms' tumor with intracardiac extension requires careful preoperative diagnosis and management. Advanced surgical techniques, including cardiopulmonary bypass and preoperative chemotherapy, improve outcomes for this rare pediatric cancer presentation.
Area of Science:
- Pediatric Oncology
- Cardiovascular Surgery
Background:
- Wilms' tumor (WT) is a rare pediatric kidney cancer.
- Intracardiac extension of WT is a rare but serious complication.
Observation:
- This study reviews 4 cases of WT with intracardiac extension.
- Patients underwent cardiopulmonary bypass with circulatory arrest and profound hypothermia.
Findings:
- No surgical complications were observed.
- Preoperative diagnosis using Doppler ultrasound is crucial for all WT cases.
- Preoperative chemotherapy may reduce thromboembolism risk during surgery.
Implications:
- Patients with intracardiac WT require specialized multidisciplinary care.
- Intracardiac extension does not worsen prognosis with appropriate surgical management.
- Doppler ultrasound and preoperative chemotherapy are recommended for managing WT with intracardiac extension.
Abstract:
We review our experience and the literature in treating 4 patients with Wilms' tumor (WT) with intracardiac extension among 92 patients with this neoplasm. Cardiopulmonary bypass with circulatory arrest and profound hypothermia was used. There were 3 boys (3 years, 4 years 5 months, and 15 years) and 1 girl (6 years). The follow-up periods were 8 months, 3 years, 2 years 6 months, and 15 years, respectively. We had no surgical complications and conclude that the preoperative diagnosis is extremely important. These patients must be transferred to institutions where concomitant cardiac procedures can be performed. In treating patients with WT, Doppler ultrasound must be used preoperatively in all cases, not only those in which clinical and radiologic signs of intravascular involvement are found. We propose that preoperative chemotherapy should be used, as it shrinks the thrombus and causes desirable adherence of the thrombus to the venous wall, reducing the probability of thromboembolism during the surgical procedure. We also find this method safer than in our 1st case, where neither cardiac arrest nor hypothermia was used. Our results agree with the literature that intracardiac extension of WT does not worsen its prognosis when a rational surgical approach is used.