Journal Article

Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights

Martelli, Helene, Pasqualini, Claudia, Abib, Simone, Guilhen, José Cícero S., Schleiermacher, Gudrun, Le Bret, Emmanuel, De Souza, Fernanda K. M., Rod, Julien, Tamisier, Daniel, Chardot, Christophe, Fanelli, Mayara C. A., Blanc, Thomas, Cypriano, Monica D. S., Belli, Emré, Fadel, Elie, Irtan, Sabine, Sarnacki, Sabine, Gauthier, Frederic, Guerin, Florent, Lemelle, Lauriane, Pistorio, Angela, Minard, Véronique, Sarrai, Nadia, Branchereau, Sophie, Pio, Luca

2024

Overview

Background About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. Patients and Methods A retrospective study of pediatric Wilms tumors treated at three sites (January 1999–June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. Results Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd–Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival ( p = 0.01). With a median follow-up of 9 years (range: 0.5–20 years), overall survival was 89% and event-free survival was 78%. Conclusions Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

• Publisher: Springer International Publishing,Springer Nature B.V
• Subject: Blood clots / Chemotherapy / Child / Child, Preschool / Diagnosis / Embolism / Female / Follow-Up Studies / Heart Atria - pathology / Heart Atria - surgery / Humans / Infant / Kidney Neoplasms - pathology / Kidney Neoplasms - surgery / Kidneys / Male / Medical prognosis / Medicine / Medicine & Public Health / Neoadjuvant Therapy / Nephrectomy / Oncology / Patients / Pediatric Oncology / Pediatrics / Prognosis / Renal insufficiency / Renal Veins - pathology / Renal Veins - surgery / Retrospective Studies / Risk factors / Surgery / Surgical Oncology / Survival / Survival Rate / Thrombosis / Tumors / Vena Cava, Inferior - pathology / Vena Cava, Inferior - surgery / Wilms Tumor - pathology / Wilms Tumor - surgery