Wilms Tumor (Renal CA) Flashcards
1
Q
Wilms Tumor
A
- one of the most common primary neoplasms of younger children (~2-5 yrs)
2
Q
associated congenital abnormalities
A
- aniridia
- hemihypertrophy
- anomalies of GI system
3
Q
aniridia
A
absence of iris
4
Q
hemihypertrophy
A
enlargement of 1 side of face/body
5
Q
chromosomal abnormalities
A
one Wilms Tumor gene (WT1 on Chr11)
6
Q
WT1 gene
A
encodes transcription factor critical for normal kidney development
(~10-20% of all Wilms Tumors have mutated WT1)
7
Q
tumor characteristics
A
- sharply defined & encapsulated
- occurs in any part of kiney
- grows to any size
- distorts kidney structure
8
Q
how many stages
A
4
9
Q
stage I
A
limited to kidney & can be excised w/ capsular surface intact
10
Q
stage II
A
extends into renal capsule, can be excised
11
Q
stage III
A
extension of tumor occurs but is confined to abdo
12
Q
stage IV
A
metastasis (commonly to lung via blood)
13
Q
mnfts
A
- largely asympt abd mass
- HTN
- some children may have abd pain, vomiting, or gross hematuria
14
Q
Dx
A
CT scan
15
Q
Tx
A
- Sx
- chemo
- radiation (sometimes)