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