Cancer

Question 1

Kidney + brain tumour

Answer 1

ATRT (loss of SMARC/INI1)

Question 2

Kidney + skeletal mets

Answer 2

clear cell sarcoma

Question 3

Kidney + lung

Answer 3

wilms

Question 4

Kidney + IVC clots

Answer 4

wilms

Question 5

Wilms treatment

Answer 5

dactinomycin + vincristine
- doxorubicin if advanced disease
- if lung mets go within 6 months of chemo, no radiation required
90% survival

Question 6

Wilms predisposition

Answer 6

problems with kidneys and genital malformation

Question 7

WAGR syndrome

Answer 7

WT1 deletion, 11p13
wilms, aniridia (PAX6 del), GU, retardation

Question 8

Denys-Drash

Answer 8

WT1 missense
intersex, nephropathy, wilms

Question 9

Familial Wilms

Answer 9

FWT1 and 2 (17 and 19)

Question 10

Ewings location

Answer 10

diaphysis (e close to d) i,.e. middle of bones
also pelvis
BONE AND SOFT TISSUE

Question 11

Ewings appearance

Answer 11

lytic, onion skin

Question 12

Ewings mets

Answer 12

lungs, bone , marrow

Question 13

Ewings genetics

Answer 13

t(11;22) in 90%

Question 14

Ewings Rx

Answer 14

chemo, surgery, radiation

Question 15

Osteosarcoma location

Answer 15

metaphysis, knee, prox humerus

Question 16

Osteosarcoma appearance

Answer 16

sunburst, sclerotic

Question 17

Osteosarcoma mets

Answer 17

lungs, bones

Question 18

Osteosarcoma genetics

Answer 18

predisposition retinoblastoma, TP53, prior radio

Question 19

Osteosarcoma Rx

Answer 19

surgery and chemo, no radiotherapy

Question 20

Sarcoma mets to marrow

Answer 20

Ewings and alveolar rhabdomyosarcoma (not embryonal rhabdomyo)