Wilms

Question 1

Inheritance

Answer 1

Mostly sporadic, 1-2% inherited AD variable pen

Question 2

10% of Wilms ass with other conditions

Name 3 overgrowth syndromes and the associated gene

Answer 2

Alpha WT2
Beckwith Wiedemann
Sotos (cerebral gigantism)
Isolated hemihypertrophy

Question 3

Name non overgrowth syndromes ass with Wilms

Answer 3

Denys Drash (WT1 gene)
- male pseudohermaphroditism (46xy)
- renal imp in first year
- Mesangial nephropathy
WAGR (Wilms 30% risk, Aniridia, Genitourinary, mental Retardation)
Trisomy 18 Edwards
Urogenital malf - cryptorchidism, renal fusion, hypospadius

Question 4

WT2 which chrom?

Answer 4

chr 11q15 neighbouring gene responsible for BWS

Think overgrowth syndromes

Question 5

WT1 gene ? Which chrom

Answer 5

Chr 11p13

A/w Aniridia, Genitourinary malf, only in 5-10% of Wilms Tumours

Question 6

Which gene defect is associated with anaplastic poor prognostic Wilms?

Answer 6

P53 (85% of anaplastic Wilms)

Question 7

How many have mets at dx and where

Answer 7

10-15%

LN, LUNG (80%), LIVER

Question 8

Age of kids

Answer 8

2-5y (80%

Question 9

Common Chemo used?

Answer 9

Vin/Actinomycin/Doxo

Shrinks well then more amenable to surgery