Wilms Tumor

Question 1

What is Wilms Tumor

Answer 1

It is the most common renal tumor of childhood due to inactivation of WT1 gene.
Second most common abdominal tumor

Question 2

Symptoms

Answer 2

Palpable abdominal mass
Hematuria

Decreased appetite
Nausea

Question 3

Which sex and race is more predominant in

Answer 3

Females, African Americans

Question 4

Median age

Answer 4

3-4 years

Question 5

B/L Wilms is seen in

Answer 5

younger population, 2.5 yrs

Question 6

Predisposing factors

Answer 6

Congenital abnormalities
Dad: Welder/works with machines
Mom: Using hair dyes

Question 7

What congenital abnormalities

Answer 7

WAGR
Denys-Drash
Beckwith-weidemann syndrome

Question 8

What is WAGR

Answer 8

11p13

W: Wilms tumor
A: Aniridia
G: Growth retardation
R: Renal abnormalities

Question 9

What is Denys- drash

Answer 9

Wilms tumor
early kidney failure
ambiguous genitalia

Question 10

What is Beckwith-weidemann syndrome

Answer 10

11p15.5

Wilms tumor
Enlarges tongue
Omphalocele
Hyper insulin
Hemihypertrophy

Question 11

Labs

Answer 11

Urine Catecholamines to R/O Neuroblastoma
CBC, CMP, LFT, UA

Question 12

Imaging

Answer 12

US Abdomen: Does not cross the midline; blends in with the kidney and cannot differentiate from kidney. Lobulated and solid. NO calcifications. Usually very large tumors with compression of adjacent structures.
MRI with contrast
CT Chest: Lung Mets?

Question 13

Biopsy

Answer 13

Only done if
- B/L
- Not sure of diagnosis
- Unresectable

Question 14

Histology

Answer 14

Favorable
Unfavorable

Question 15

Favorable Histology

Answer 15

90-95%
No anaplasia or sarcomatous features
With or without 1p/16q loss

Question 16

Unfavorable Histology

Answer 16

SARC Features
S: Sarcomatous
A: Anaplastic
R: Rhabdoid tumor of Kidney
C: Clear cell tumor of Kidney

Question 17

Staging

Answer 17

Stage I: R0 resection, limited to kidney, intact capsule, LN-ve
Stage II: R0 resection, Capsule broken, Invasion into vessels
Stage III: BURP S
B: Biopsy
U: Unresectable
R: Rupture
P: Peritoneal implant, piece meal resection
S: Spillage, Subtotal resection

Stage IV: Distant mets or LN +ve
Stage V: B/L Wilms tumor

Question 18

Risk Groups

Answer 18

Very low risk
Low risk
Std Risk
High risk

Question 19

Treatment Paradigm

Answer 19

Radical Nephrectomy -> XRT -> Chemotherapy

Question 20

Surgery

Answer 20

Radical Nephrectomy
Avoid tumor spillage
If B/L : Nephron sparing surgery

Question 21

Radiation

Answer 21

For Stage III Pts
Diffuse anaplasia (unfavorable prognostic feature)
RTK: Rhabdoid tumor of kidney
CCSK:

Question 22

Radiation Dose

Answer 22

Given with concurrent Chemotherapy (Vincristine)
Should start 10-14 days post SX; ideally~ 9days

Flank: 10Gy in 6 fractions
Lymph Nodes: 10.8Gy/6fx; if unresected 19.8Gy/11fractions
WART: 10.5Gy/7fx
WLI: 12Gy/8fx
WBRT: 21.6Gy/12Fx
Bone: 25.2Gy/14fx

Question 23

Chemotherapy

Answer 23

VA x 18 weeks
V: Vincristine
A:Dactinomycin

VAC x 24 weeks for pts who have loss of heterozygosiy of 1p16q
V:VIncristine
A: Dactinomycin
C: Doxorubicin

Question 24

Toxicity

Answer 24

DM if pancreatic tail >10Gy
RT to Flank: Pregnancy issues: HTN, fetal malposition, lbw, premature birth
end stage renal disease
scoliosis at 15yr >24Gy

Question 25

4 Year OS

Answer 25

> 90%

Stave IV/V with lung mets, unfavorable histology = 30-50%

Question 26

4 Years Event free survival

Answer 26

> 80%
Stage IV/V with lung mets, unfavorable histology = 20-30%