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Surgical management of Wilms tumor with intravascular extension: a single-institution experience
Diego Aspiazu1, Israel Fernandez-Pineda, Rosa Cabello
1Department of Pediatric Surgery, Virgen del Rocio Children's Hospital, Seville, Spain.
Insights
Neoadjuvant chemotherapy effectively reduced tumor thrombus in children with Wilms tumor (WT) and intravascular extension, facilitating surgical resection. Cardiopulmonary bypass was crucial for extensive cases involving the right atrium.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Nephrology
Background:
- Wilms tumor (WT) with intravascular extension presents unique surgical challenges.
- Management strategies require careful consideration of tumor thrombus extent.
Purpose of the Study:
- To analyze the clinical presentation, treatment, and outcomes of pediatric patients with Wilms tumor and intravascular extension.
- To evaluate the effectiveness of neoadjuvant chemotherapy and surgical interventions.
Main Methods:
- Retrospective review of medical records from 1992-2010.
- Analysis of 7 pediatric patients with Wilms tumor and intravascular extension.
- Utilized chemotherapy (SIOP 2001 protocol) followed by surgical resection (nephrectomy and thrombectomy).
- Diagnostic imaging included CT, ultrasonography, and echocardiography.
Main Results:
- All 7 patients had tumor thrombus extending to the right atrium (RA) or inferior vena cava (IVC).
- Neoadjuvant chemotherapy led to regression of intravascular extension in all patients.
- Surgical intervention, including cardiopulmonary bypass for RA/suprahepatic IVC thrombus, was successful.
- All patients achieved disease-free status with long-term follow-up.
Conclusions:
- Neoadjuvant chemotherapy is beneficial for reducing tumor thrombus extent in Wilms tumor with intravascular extension.
- Cardiopulmonary bypass is indicated for cases with right atrial or suprahepatic IVC involvement.
- Accurate diagnostic imaging is essential for planning treatment and follow-up.
Abstract:
The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.4 years, range 2-8.1 years) were identified. At diagnosis, 6 of the 7 patients (85.7%) presented with tumor thrombus that reached the right atrium (RA) and 1 patient with infrahepatic inferior vena cava (IVC) thrombus. All patients received neoadjuvant chemotherapy (SIOP 2001 protocol) with vincristine, doxorubicin, and actinomycin D. Regression of the intravascular extension of the tumor was documented in all patients. Postchemotherapy level of extension was suprahepatic IVC in 1 patient, infrahepatic IVC in 2 patients, renal vein (RV) in 1 patient, and RA in 3 patients. Nephrectomy and thrombectomy were performed in all cases, requiring cardiopulmonary bypass for the 4 patients who presented with suprahepatic IVC and RA thrombus. The other 3 patients with infrahepatic IVC and RV involvement underwent cavotomy and thrombus extraction. Computed tomography, ultrasonography, and echocardiography were used for diagnosis and follow-up. All patients remain disease-free with a median follow-up of 6.3 years (range, 2-19 years). Neoadjuvant chemotherapy for WT with intravascular extension may facilitate the resection by decreasing the extent of the tumor thrombus. Cardiopulmonary bypass is indicated for suprahepatic IVC and RA involvement. Accurate diagnostic imaging is necessary.
