Peds Tumors

Question 1

What is WAGR syndrome?

Answer 1

WT-1 11p13
Wilms, Aniridia, GU malformation (hypospadias, UDT, streak gonads), intellectual disability
RBUS q3mo

Question 2

What is Denys-Drash syndrome?

Answer 2

WT-1
1) Wilm’s (90%),
2) gonadal dysgenesis (risk of gonadoblastoma) or male pseudohermaphroditism (hypospadias/orchiopexy)
3) nephropathjy (diffuse glomerulosclerosis)
Ppx bilateral nephrectomy

Question 3

What is Wilm’s tumor staging

Answer 3

1 limited to kidney, completely excised
2 extends beyond kidney but completely excised
3 residual tumor in abdomen (positive margins)
4 hematogenous/distant spread
5 bilateral renal involvement, or seeding (ie s/p bx)

Question 4

What are characteristics of neuroblastoma?

Answer 4

Arises from adrenal medulla or anywhere along sympathetic chain
<4yrs old
Pushes kidney away, irregular border, calcification, crosses midline

Question 5

What are characteristics of congenital mesoblastic nephroma?

Answer 5

Most common solid renal tumor in neonates and infants (before age 1, mean age 3mos)
Benign, but locally invasive
Well-defined mass with presence of concentric hypoechoic rings on US
Treated with nephrectomy alone, no surveillance
Do not biopsy

Question 6

How is tumor spillage of Wilm’s tumor treated?

Answer 6

favorable: vincristine, doxorubicin, dactinomycin + XRT

Unfavorable: vincristine, doxorubicin, cyclophosphamide, etoposide