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Intravascular extension of Wilms tumor
R C Shamberger1, M L Ritchey, G M Haase
1Department of Surgery, Children's Hospital, Boston, Massachusetts 02115, USA. shamberger@a1.tch.harvard.edu
Insights
Preoperative therapy for children with Wilms tumor extending into the inferior vena cava (IVC) or atrium may aid resection. However, the overall complication rate remains similar compared to primary surgery.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Nephrology
Background:
- Wilms tumor is a common pediatric kidney cancer.
- Intravascular extension into the inferior vena cava (IVC) or atrium presents unique surgical challenges.
Purpose of the Study:
- To determine the incidence and clinical features of Wilms tumor with intravascular extension.
- To evaluate the optimal therapeutic strategies for these complex cases.
Main Methods:
- Retrospective review of children in a collaborative Wilms tumor study with intravascular extension.
- Analysis of surgical checklists and pathology reports to assess tumor extent and treatment outcomes.
Main Results:
- 165 of 2,731 patients had intravascular extension (IVC: 134, Atrium: 31).
- Preoperative therapy in 69 patients showed tumor regression, potentially avoiding cardiopulmonary bypass.
- Overall complication rates were similar between preoperative therapy and primary resection groups, despite initial chemotherapy complications.
Conclusions:
- Preoperative treatment can reduce tumor thrombus extent, facilitating surgical resection.
- The overall complication incidence is comparable between primary resection and preoperative therapy groups for Wilms tumor with intravascular extension.
Objective:
To define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms tumor.
Methods:
Children on a collaborative study of Wilms tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists and surgical and pathology reports were reviewed.
Results:
One hundred sixty-five of 2,731 patients had intravascular extension of Wilms tumor. The level of extension was IVC in 134 and atrium in 31. Sixty-nine had received preoperative therapy (55 with IVC extension and 14 with atrial extension) for a median of 8 weeks. Complications during preoperative chemotherapy were seen in five patients (tumor embolism and tumor progression in one each, and three with adult respiratory distress syndrome, one of which was fatal). The intravascular extension of the tumor regressed in 39 of 49 children with comparable pre- and posttherapy radiographic studies, including 7 of 12 in whom the tumor regressed from an atrial location, thus obviating the need for cardiopulmonary bypass. Surgical complications occurred in 36.7% of the children in the atrial group and 17.2% in the IVC group. The frequency of surgical complications was 26% in the primary resection group versus 13.2% in children with preoperative therapy. When all the complications of therapy were considered, including those that occurred during the interval of preoperative chemotherapy (one of the five also had a surgical complication), the incidence of complications among those receiving preoperative therapy was not statistically different from the incidence among those who underwent primary resection. The difference in 3-year relapse-free survival (76.9% for 165 patients with intravascular extension, 80.3% for 1,622 patients with no extension) was not statistically significant whether or not it was adjusted for stage and histology.
Conclusions:
Preoperative treatment of these children may facilitate resection by decreasing the extent of the tumor thrombus, but the overall frequency of complications is similar in both groups.
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