Wilms tumor is a malignant tumor of the kidney occurring in the pediatric age group. It is also referred to as nephroblastoma.
Slightly more common in girls than boys; 90% of patients present by age 6 years
1 in 10,000 live births
- Most common primary malignant renal tumor of childhood
- 5–6% of all childhood cancers
- 5–10% present in the context of a predisposing syndrome
- Familial cases represent 1–2% of cases and are more often bilateral and occur at an earlier age.
- A tumor suppressor gene related to Wilms tumor (WT1) has been localized to chromosome 11p13. Mutations in this gene occur in 10–20% of Wilms tumors.
- Another tumor suppressor gene, WT2, has been localized on 11p15.
- 10–20% of Wilms tumors have a mutation in the WT1 tumor suppressor gene.
- Causes in the remaining 80% of patients are complex and involve the WNT signaling pathway and IGF2 overexpression.
Commonly Associated Conditions
- 12–15% of patients have other congenital anomalies.
- May be associated with aniridia, hemihypertrophy, and cryptorchidism
- Associated syndromes
- WAGR (Wilms tumor, aniridia, genitourinary [GU] abnormalities, mental retardation)
- Beckwith-Wiedemann syndrome (macroglossia, omphalocele, visceromegaly, hemihypertrophy)
- Denys-Drash syndrome (ambiguous genitalia, progressive renal failure, and increased risk of Wilms tumor)
- Nephroblastomatosis is a condition where islands of embryonic renal tissue develop. This is a generally benign condition which carries a risk of developing into Wilms tumor.
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