kidney tumors

Question 1

3 genetic changes that are bad for pts with wilms

Answer 1

TP53, LOH 1p/16q, gain of 1q

Question 2

what does TP53 correlate with in wilms

Answer 2

anaplasia

Question 3

how many pts with wilms have genetic predisposition

Answer 3

33%

Question 4

most common loci of genetic changes for wilms

Answer 4

11p15 (BWS)

Question 5

most common mets for wilms

Answer 5

1) lung 2) liver

Question 6

3 chemo drugs for wilms

Answer 6

VCR, dactinomycin, doxo

Question 7

define very low risk wilms

Answer 7

< 2 years old, <550 g, stage 1, favorable histology – only surgery needed

Question 8

adult renal cell carcinoma- what type and what genetic syndrome

Answer 8

clear cel, VHL

Question 9

what is the genetic change in kids with renal cell carcinoma

Answer 9

translocation histology- TFE3 translocations (Xp11)

Question 10

sickle cell trait risk for renal cancer

Answer 10

renal medullary carcinoma

Question 11

most common genetic changes in clear cell sarcoma of the kidney

Answer 11

BCOR internal tandem duplications and t(1017)

Question 12

where does clear cell sarcoma of the kidney metastasize to

Answer 12

brain and bone

Question 13

fusion for congenital mesoblastic nephroma

Answer 13

same as infantile fibrosarcoma- t(12;15) ETV- NTRK3