Peds Renal Tumors

Question 1

Most common primary malignant renal tumor in children

Answer 1

Wilms Tumor

Question 2

Average age of presentation for Wilms

Answer 2

3.5-4 years old.
If bilateral, typically earlier presentation (2.5 years old)
Rare to present in newborns

Question 3

Presenting characteristics of Wilms

Answer 3

Abdominal mass +/- HTN, microhematuria.
Rarely crosses midline.

15% present with other congenital anomalies
20% present with gross renal vein invasion
5-10% present with acquired von Willebrand disease

Question 4

Mutations linked to Wilms

Answer 4

WT1 (11p13)
WT2 (11p15)

inactivation of these genes leads to Wilms
16q loss of heterozygosity also leads to Wilms

Question 5

Metastatic behavior of Wilms

Answer 5

pulmonary, hepatic
Rare: bone/brain