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Background: Primary surgical management of Wilms tumor with intravascular tumor extension is associated with significant morbidity. Retrospective analysis of a group of Wilms tumor patients with intravascular tumor extension treated with preoperative therapy was performed to evaluate the feasibility of this approach., Methods: Thirty children with caval (15 patients) or atrial (15 patients) extension of Wilms tumor who received preoperative therapy were reviewed. Patients were classified according to the stage of the disease as follows: (1) Stage III, 7 patients; (2) Stage IV, 18 patients; and (3) Stage V, 5 patients. The histologic type was favorable in 20 patients, anaplastic Wilms tumor in 7, and unknown in 3. All of the children received preoperative chemotherapy, ranging from 1-16 weeks with an average of 7.2 weeks., Results: One child died of progressive disease and another from toxicity before surgery. In 23 patients, a decrease in the size of the intravascular extension was noted preoperatively, without tumor embolization in any case. There was complete resolution of tumor thrombus in seven patients. Two-year survival for the entire group is 70%, with a median follow-up period of 21 months., Conclusions: The use of preoperative therapy is appropriate in selected patients with extensive intravascular tumor involvement. Surgical management was facilitated in the majority of cases due to shrinkage of the primary tumor and reduction in the size of the tumor thrombus.

A comprehensive statistical analysis of relapses to lung and abdomen and of deaths due to tumor that occurred among 1466 patients with nonmetastatic, favorable histology (FH) Wilms' tumor entered on the Third National Wilms' Tumor Study (NWTS-3) was undertaken. In addition to lymph node involvement, age at diagnosis and tumor size as measured by the weight of the excised specimen were the most important determinants of outcome. The effects of tumor size were most apparent for patients with localized (Stage I) disease; age effects were found for patients with regional (Stage II or III) disease. The presence of microscopic tumor in the margin of surgical resection was an independent predictor of abdominal recurrence and death in the latter group. Although the report of the surgeon of diffuse soilage of the peritoneal cavity from tumor spilled or cut across in the course of nephrectomy was highly correlated with outcome, it was not possible to establish an independent prognostic role for such a finding after adjustment for the aforementioned factors. Patients with intralobar nephrogenic rests (ILNR) had a favorable survival outlook even after accounting for their generally younger ages and lower stages.