Pediatric Renal Tumors Flashcards
What is the incidence of Wilms tumor in children in North America?
Roughly 500 cases of Wilms tumor occur in North America per year, giving an incidence of roughly 8 per 1,000,000. Wilms tumor is the most common malignant pediatric renal tumor, and accounts for 7% of all childhood cancers.
How often are congenital anomalies associated with Wilms tumor?
Fifteen percent of cases. Congenital anomalies are much more common in patients with bilateral or simultaneous multifocal tumors.
What are the major Wilms tumor study groups?
In North America, the renal committee of the Children’s Oncology Group (COG) is the major cooperative Wilms tumor study group. In Europe, the International Society of Pediatric Oncology (SIOP) is the major cooperative Williams tumor study group.
What is the relationship of aniridia to Wilms tumor?
Aniridia is caused by an abnormality of the PAX6 gene located adjacent to WT1, and is seen in 1% of patients with Wilms tumors. Of patients with aniridia, 40% of those having a chromosomal deletion that includes the locus for WT1 will develop Wilms tumor. Conversely, those with a normal WT1 do not develop Wilms tumor.
