From the Guidelines
Wilms tumor is a rare kidney cancer with a prevalence that is not explicitly stated in the provided evidence, but it is known to primarily affect children, accounting for about 5-6% of all childhood cancers. The provided evidence 1 discusses kidney cancer in general, stating that approximately 85% of kidney tumors are renal cell carcinoma (RCC), with about 70% being of clear cell histology, but it does not specifically address Wilms tumor prevalence. However, based on general medical knowledge, Wilms tumor typically occurs in children under the age of 5, with the peak incidence around 3-4 years of age. Some key points about Wilms tumor include:
- It is the most common type of kidney cancer in children
- The prevalence varies slightly by ethnicity
- There is a slight gender difference, with girls having a marginally higher risk than boys
- About 5-10% of cases are associated with specific genetic syndromes
- Most cases (about 90%) are sporadic with no clear genetic cause
- Early detection is crucial for successful treatment, as Wilms tumor has an excellent prognosis when diagnosed early, with overall survival rates exceeding 90% with appropriate treatment combining surgery, chemotherapy, and sometimes radiation therapy, as supported by the importance of early diagnosis and treatment in improving survival rates for kidney cancer patients in general, as indicated by the analysis of the SEER database 1.
From the Research
Wilms Tumor Prevalency
- Wilms tumor is the most common primary malignant renal tumor of childhood, with approximately 500 new cases diagnosed in the United States every year 2.
- It is the fifth most common pediatric malignancy, arising from the embryonal tissue of kidneys 3.
- The current survival rate exceeds 80%, primarily due to large multi-institutional trials such as the National Wilms' Tumor Study (NWTS) 3.
- Advances in multimodal therapy including surgery, chemotherapy, and radiation therapy given according to risk stratification have allowed most patients to achieve survival rates in excess of 90% 2.
Incidence and Survival Rates
- The two-year relapse-free survival (RFS) and two-year survival rates were 88 and 95%, respectively, for patients with tumors that were confined to the kidney and that had been totally excised 4.
- Survival rates at two years were 54% for unfavorable histology (UH) vs. 90% for favorable histology (FH) 4.
- Four-year event-free survival (EFS) and overall survival (OS) were 42.3 and 48.0% respectively for all patients who relapsed after initial treatment with VAD and radiation therapy 5.
- Five-year EFS and OS were 79% and 90%, respectively, for patients with clear cell sarcoma of the kidney (CCSK) treated on the National Wilms Tumor Study-5 (NWTS-5) 6.