PAEA Surgery End Of Rotation (EOR) Practice Exam

Which syndrome is commonly associated with Wilm's tumor?

1. Down syndrome

2. Beckwith-Wiedemann syndrome

3. Marfan syndrome

4. Turner syndrome

The correct answer is: Beckwith-Wiedemann syndrome

Wilm's tumor, a pediatric kidney cancer, has a well-established association with Beckwith-Wiedemann syndrome. This is a genetic disorder characterized by overgrowth and an increased risk of tumors, among other symptoms. The connection between Beckwith-Wiedemann syndrome and Wilm's tumor is significant; children with this syndrome have a higher incidence of renal tumors, particularly Wilm's tumor, due to the dysregulation of growth factors involved in kidney development. In the context of the other options, while there may be associations with certain conditions, they do not exhibit the same level of direct connection to Wilm's tumor as Beckwith-Wiedemann syndrome does. Therefore, the recognition of this specific genetic syndrome as a primary risk factor for Wilm's tumor is critical for both diagnosis and management in pediatric patients with concerning abdominal masses.