Ruptured Wilms Tumor: Clinical Features, Diagnostic Challenges, and Survival Outcomes

Wilms tumor (WT) rupture is a serious complication that upstages the disease and requires treatment intensification. This study evaluates clinical characteristics, radiological-pathological concordance, and survival outcomes of ruptured versus non-ruptured WT at a major Middle Eastern tertiary center.

We conducted a retrospective cohort study of 111 pediatric patients with unilateral WT treated at King Hussein Cancer Center, Jordan, between October 2014 and December 2023 (follow-up to December 2025). Tumor rupture was defined by preoperative CT findings (peritumoral effusion, hemorrhage, or peritoneal nodules), intraoperative capsular breach/spillage, or pathological confirmation. Event-free survival (EFS) and overall survival (OS) were estimated using Kaplan-Meier methods and compared with the log-rank test. Multivariable Cox regression identified independent prognostic factors.

Tumor rupture occurred in 17 patients (15.3%). Ruptured cases were older (median 4.2 vs. 3.5 years, p = 0.03), had larger tumors (13.7 vs. 11.7 cm, p = 0.01), and presented with lower hemoglobin (7.9 vs. 10.4 g/dL, p < 0.001). All ruptured cases were stage III/IV, with 41% having distant metastases at diagnosis. Five-year EFS was 44.1% vs. 75.8% (p = 0.025) and OS was 58.2% vs. 81.4% (p = 0.002) for ruptured vs. non-ruptured groups. On multivariable analysis, rupture independently predicted death (HR 17.62, 95% CI 2.69-115.48, p = 0.003) and relapse (HR 8.1, 95% CI 1.66-39.57, p = 0.01).

WT rupture is associated with advanced disease at presentation and significantly inferior survival. Substantial discordance between preoperative radiological/intraoperative findings and post-chemotherapy pathology highlights the "masking effect" of neoadjuvant chemotherapy. A multidisciplinary approach integrating initial imaging, surgical notes, and histology is essential to avoid undertreatment in SIOP-based protocols.