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Background Wilms tumor (WT) is the most common malignant renal tumor in children. This study investigated the clinical features, pathological findings, and outcomes of children with malignant renal tumors in Southern Iran. Factors associated with recurrence and mortality were assessed. Methods Electronic files of children with malignant renal tumors from 2009 to 2023 were reviewed. The 5-year overall survival (OS) and event-free survival (EFS) were reported. Results Eighty-three patients (44 males) with a median age of 40 months (range: 3-122) were included. WT was the most common pathological variant (94%). Anaplasia was found in 17.3% of patients. Upfront chemotherapy followed by nephrectomy was performed in 54.2% of the patients. Ten patients (12%) experienced relapse, and five patients (6%) died during the 14-year follow-up. The 5-year OS and EFS were 90.75% (95% CI, 78.64–96.16%) and 81.9% (95% CI, 70.10-89.38%), respectively, and were comparable between the two treatment strategies (upfront chemotherapy vs. upfront nephrectomy). Metastasis and residual disease were associated with relapse, whereas tumor recurrence was the only predictive factor of survival. Conclusion WT is a curable disease with excellent outcomes if diagnosed and treated promptly. The timing of nephrectomy does not affect OS and EFS. Patients with low-stage tumors and those with complete surgical excision are at a lower risk of tumor recurrence. Relapse is the primary risk factor for death. Supplementary Information The online version contains supplementary material available at 10.1186/s12885-024-13150-4.
Background Wilms tumor (WT) is the most common malignant renal tumor in children. This study investigated the clinical features, pathological findings, and outcomes of children with malignant renal tumors in Southern Iran. Factors associated with recurrence and mortality were assessed. Methods Electronic files of children with malignant renal tumors from 2009 to 2023 were reviewed. The 5-year overall survival (OS) and event-free survival (EFS) were reported. Results Eighty-three patients (44 males) with a median age of 40 months (range: 3-122) were included. WT was the most common pathological variant (94%). Anaplasia was found in 17.3% of patients. Upfront chemotherapy followed by nephrectomy was performed in 54.2% of the patients. Ten patients (12%) experienced relapse, and five patients (6%) died during the 14-year follow-up. The 5-year OS and EFS were 90.75% (95% CI, 78.64–96.16%) and 81.9% (95% CI, 70.10-89.38%), respectively, and were comparable between the two treatment strategies (upfront chemotherapy vs. upfront nephrectomy). Metastasis and residual disease were associated with relapse, whereas tumor recurrence was the only predictive factor of survival. Conclusion WT is a curable disease with excellent outcomes if diagnosed and treated promptly. The timing of nephrectomy does not affect OS and EFS. Patients with low-stage tumors and those with complete surgical excision are at a lower risk of tumor recurrence. Relapse is the primary risk factor for death. Supplementary Information The online version contains supplementary material available at 10.1186/s12885-024-13150-4.
Background/Objectives: Wilms tumor (WT) is the most common form of pediatric renal tumor, accounting for over 90% of cases followed by hypernephroma. Some pediatric patients with WT (10%) experience relapse or metastasis and have poor survival rates. PD-L1 assists cancer cells in escaping damage from the immune system. P53 mutations are found in relapsed WT tumor samples. We hypothesized that testing circulating PD-1 and PD-L1 and P53 expression levels could offer a simple method to predict patient relapse and explore novel treatments for pediatric WTs and hypernephroma. Methods: Flow cytometric detection of cPD-1, cPD-L1, and P53 expression in relapsed and in-remission WT and hypernephroma before and after one year of chemotherapy was performed. Results: Our data shows increased levels of cPD-L1 in relapsed pediatric patients with WT or hypernephroma before and after chemotherapy. There were also slight and significant increases in cPD-1 levels in relapsed groups before chemotherapy. Additionally, we observed significant decreases in P53 expression after one year of chemotherapy in relapsed pediatric patients. Conclusions: Our study found that circulating PD-L1 can be used as a predictor marker for WT and hypernephroma relapse. In conclusion, these circulating markers can assist in monitoring relapse in WT and hypernephroma patients without the need for several biopsies.
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