Clinical and prognostic characteristics of bilateral Wilms tumor: a multi-center institutional retrospective cohort study experience from China.

BACKGROUND

This multi-center analysis of data from China reviews the management and long-term outcomes of patients with bilateral Wilms tumors (BWT), and explores prognostic risk factors.

METHODS

We retrospectively analyzed a cohort of pediatric patients with synchronous BWT treated at 18 pediatric oncology centers in China between 2006 and 2023. The overall survival (OS) and event-free survival (EFS) rates were calculated using Kaplan-Meier methodology. Prognostic risk factors were determined using univariable and multivariable analysis.

RESULTS

A total of 167 patients with BWT and a median age at diagnosis of 13 months (range 0-78 months) were included in the study. Neoadjuvant chemotherapy was administered to 149 index cases; tumor biopsy was performed before initiating chemotherapy in 70 patients. One hundred and three children underwent bilateral nephron-sparing surgery (NSS) and two hundred fifty-two of the three hundred one kidneys underwent NSS. The four-year OS and EFS rates in the study cohort were 86.5% and 77.8%. After a median follow-up of 50 months, four patients developed renal failure requiring dialysis and a single patient received a kidney transplant 26 months postoperatively.

CONCLUSIONS

Regarding prognostic factors, the results of the multifactor analysis indicate that distant metastasis and positive surgical margins have negative impacts on OS and distant metastasis had a negative effect on EFS. Distant metastasis and positive surgical margins affect the long-term prognosis of BWT. Video Abstract (MP4 19393 KB).