Bilateral Wilms Tumor: Looking for the Best Surgical Strategy.

Abstract

Management of bilateral Wilms tumor (BWT) to achieve complete tumor control and maintain good long-term renal function is challenging. Nephron-sparing surgery (NSS) allows preservation of renal parenchyma and reduces the risk of renal failure in selected cases. However, there is no standardized surgical strategy. This study analyzed the surgical management for patients included in the SIOP2001 trial and its outcomes. This retrospective review analyzed French patients included in the SIOP 2001 protocol for synchronous BWT. Data regarding imaging, surgical strategy, complications, histology, oncologic outcomes, and renal function were collected. The study included 96 patients (median age, 15 months; range, 0-143 months). They received surgery for 122 Wilms tumors, and 56 (45.9 %) kidneys benefited from NSS. Total nephrectomy (TN) was performed in 27/35 (77.1 %) and 33/76 (43.4 %) of the histologies non-sensitive to chemotherapy (stromal and diffuse anaplasia, respectively). For bilateral surgery, the study found a trend to more TNs, tumoral rupture, and death among the patients who had surgery in a single stage (p > 0.05). Tumor rupture was associated with higher tumoral volume (p < 0.05). In a review of preoperative images, vascular contact was the main factor associated with TN. Additional chemotherapy did not seem to significantly reduce this contact or the number of TNs in these patients. Whenever possible, NSS should be prioritized for patients with BWT. In this study, the most limiting factor was contact with vascular structures. Additional chemotherapy for tumors with non-sensitive histology or extensive vascular contact had little impact on the final surgical strategy. Staged surgery should be considered as it appeared to reduce surgical morbidity.