Wilms Tumor Flashcards
What is Wilms Tumor
It is the most common renal tumor of childhood due to inactivation of WT1 gene.
Second most common abdominal tumor
Symptoms
Palpable abdominal mass
Hematuria
Decreased appetite
Nausea
Which sex and race is more predominant in
Females, African Americans
Median age
3-4 years
B/L Wilms is seen in
younger population, 2.5 yrs
Predisposing factors
Congenital abnormalities
Dad: Welder/works with machines
Mom: Using hair dyes
What congenital abnormalities
WAGR
Denys-Drash
Beckwith-weidemann syndrome
What is WAGR
11p13
W: Wilms tumor
A: Aniridia
G: Growth retardation
R: Renal abnormalities
What is Denys- drash
Wilms tumor
early kidney failure
ambiguous genitalia
What is Beckwith-weidemann syndrome
11p15.5
Wilms tumor
Enlarges tongue
Omphalocele
Hyper insulin
Hemihypertrophy
Labs
Urine Catecholamines to R/O Neuroblastoma
CBC, CMP, LFT, UA
Imaging
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?
Biopsy
Only done if
- B/L
- Not sure of diagnosis
- Unresectable
Histology
Favorable
Unfavorable
Favorable Histology
90-95%
No anaplasia or sarcomatous features
With or without 1p/16q loss
Unfavorable Histology
SARC Features
S: Sarcomatous
A: Anaplastic
R: Rhabdoid tumor of Kidney
C: Clear cell tumor of Kidney
Staging
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
Risk Groups
Very low risk
Low risk
Std Risk
High risk
Treatment Paradigm
Radical Nephrectomy -> XRT -> Chemotherapy
Surgery
Radical Nephrectomy
Avoid tumor spillage
If B/L : Nephron sparing surgery
Radiation
For Stage III Pts
Diffuse anaplasia (unfavorable prognostic feature)
RTK: Rhabdoid tumor of kidney
CCSK:
Radiation Dose
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
Chemotherapy
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
Toxicity
DM if pancreatic tail >10Gy
RT to Flank: Pregnancy issues: HTN, fetal malposition, lbw, premature birth
end stage renal disease
scoliosis at 15yr >24Gy
4 Year OS
> 90%
Stave IV/V with lung mets, unfavorable histology = 30-50%
4 Years Event free survival
> 80%
Stage IV/V with lung mets, unfavorable histology = 20-30%
