Malignancy

Question 1

malignant childhood renal tumours

Answer 1

nephroblastoma /Wilms
renal cell cancer
clear cell sarcoma
rhabdoid renal tumour
neuroblastoma

Question 2

benign childhood renal

Answer 2

mesoblastic nephroma
angiomyolipoma
haemangioma
cystic nephroma

Question 3

DDx of chidlhood abdominal mass

Answer 3

massive HN
renal tumour
renal cysts
bowel duplication
neuroblastoma
hepatic tumour
soft tissue sarcoma

Question 4

conditions predisposing to Wilms

Answer 4

Denys Drash
WAGR
Beckwith Wiedemann

Question 5

WAGR

Answer 5

wilms
aniridia
GU malformation
mental retardation

Question 6

bilateral disease Wilms

Answer 6

5%

Question 7

peak age presentation
when do wilms present
male vs female wilms
bilateral disease

Answer 7

3-4 years
majority wilms present under age 5 75%
M and F equally affected
5%

Question 8

neuroblastoma urine test

Answer 8

urine VMA vanillylmandelic acid

Question 9

staging wilms tumour

Answer 9

1 = completely confined to kidney excised
2 =out of capsule completely excised
3= local invasion, incomplete resection, positive LN, tumour spill not confined to flank
4= metastatic disease, usually lung
5= bilateral

Question 10

prognosis wilms low risk histology 5 year survival

Answer 10

1/2 = >90
III = 70-80
IV= 60-80%
V= 70-80%

Question 11

poor prognosis histology types

Answer 11

nephroblastoma blastemal type
diffuse anaplasia type
clear cell sarcoma kidney
rhabdoid tumour kidney

Question 12

survival 5 year bad histology

Answer 12

clear cell sarcoma = 70-80%
rhabdoi 20%
anaplastic wilms 60%

Question 13

triphasic histology of Wilms

Answer 13

stromal
blastemal
epithelial / tubules

Question 14

preop chemo regimen

Answer 14

vincristine
actinomycin D
for 4 weeks then repeat imaging preop us doppler and MRI

Question 15

post op histology risk stratification

Answer 15

low risk stage 1 no further treatment
most other VA for 27 weeks
stage III need RT
High risk add doxorubicin

Question 16

mesoblastic nephroma

Answer 16

benign tumour under 6 months
homogenous non enhacing solid mass needs just primary nephrectomy
most common tumour neanatal period

Question 17

metanephric blastema

Answer 17

embronal or primitive cells give rise to renal parenchyma, if persis beyond 36/40 week called nephrogenic rests

Question 18

nephroblastomatosis

Answer 18

multifocal diffuse nephrogenic rests
appear as oval shapes in periphery
no psuedocapsle
homogenous hypoechoic

Question 19

types of RMS

Answer 19

embryonal >50% - botryoid, spindle cell paratesticular

alveolar 20%

Question 20

investigations RMS

Answer 20

pelvic and urinary tract US

Question 21

histological components Wilms

Answer 21

metanephric blastema
primitive renal tubular epithelium
connective tissue components

Question 22

mutation wilms

Answer 22

chromosome 11 mutation or deletion WT1 tumour supressor gene

Question 23

Denys Drash syndrome

Answer 23

Wilms

ambiguous genitalia

Question 24

Beckwith Wiedemann syndrome

Answer 24

Macroglossia

Question 24

associations wilms tumour

Answer 24

Denys Drash Beckwith Wiedemann Horseshoe kidney

Question 25

Rhabdomyosarcoma | organs involved

Answer 25

``` bladder base prostate paratesticular uterus vagina ```

Question 26

Rhabdomyosarcoma association

Answer 26

Li Fraumeni syndrome p53 mutation

Question 27

Rhabdomyosarcoma better vs worse prognosis form

Answer 27

embryonal forms have better prognosis, of which bladder mostly embryonal

Question 28

Neuroblastoma organ

Answer 28

arising in neuro ectoderm 50% arise adrenal rest from sympathetic chain

Question 29

Neuroblastoma median age diagnosis

Answer 29

2 years

Question 30

Neuroblastoma poor prognosis

Answer 30

deletion short arm chromosome 1

Question 31

Neuroblastoma scan

Answer 31

MIBG scan highly sensitivie

Question 32

differential abdominal mass / renal mass

Answer 32

WIlms tumours XGP usually with anaemia and listlessness ``` Benign XGP Large HN Renal cysts Splenomegaly Bowel duplication ``` Malignant Nephroblastoma Neuroblastoma Hepatic tumour Soft tissue sarcoma

Question 33

wilms 5 year survival

Answer 33

85% five year survival | if unilateral localised then prognosis in high 90s

Question 34

pain in Wilms tumour | incidence haematuria

Answer 34

Pain is uncommon and usually results from bleeding into the tumour Haematuria occurs in 10–15% of cases

Question 35

imaging vs biopsy

Answer 35

if presents in typical age 6 months to 6 years of age imaging diagnosis is sufficient

Question 36

what to look for on imaging with wilms

Answer 36

neuroblastoma encase vessels Wilms grow into vessels imaging look for tumour extension into vessels manage with nephrectomy or need vascular surgery

Question 37

staging Wilms

Answer 37

can met into lungs so need CT check

Question 38

bilateral Wilms tumour

Answer 38

stage 5 | need to think of nephron

Question 39

syndromes associated with Wilms

Answer 39

hemihypertrophy WAGR Denys Drash Beckwith Wiedemann tend to be multifocal and bilateral

Question 40

chemotherapy Wilms

Answer 40

Always give neoadjuvant chemo ``` vincristine actinomycin doxorubicin added selected cases give 6 weeks shrinks tumours reduced rate of spillage at surgery ```

Question 41

Wilms surgery

Answer 41

aim to sample LN at least 7