Malignancy Flashcards

1
Q

malignant childhood renal tumours

A
nephroblastoma /Wilms
renal cell cancer
clear cell sarcoma
rhabdoid renal tumour
neuroblastoma
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2
Q

benign childhood renal

A

mesoblastic nephroma
angiomyolipoma
haemangioma
cystic nephroma

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3
Q

DDx of chidlhood abdominal mass

A
massive HN
renal tumour
renal cysts
bowel duplication
neuroblastoma
hepatic tumour
soft tissue sarcoma
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4
Q

conditions predisposing to Wilms

A

Denys Drash
WAGR
Beckwith Wiedemann

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5
Q

WAGR

A

wilms
aniridia
GU malformation
mental retardation

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6
Q

bilateral disease Wilms

A

5%

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7
Q

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

A

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

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8
Q

neuroblastoma urine test

A

urine VMA vanillylmandelic acid

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9
Q

staging wilms tumour

A

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

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10
Q

prognosis wilms low risk histology 5 year survival

A
1/2 = >90
III = 70-80
IV= 60-80%
V= 70-80%
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11
Q

poor prognosis histology types

A

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

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12
Q

survival 5 year bad histology

A

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

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13
Q

triphasic histology of Wilms

A

stromal
blastemal
epithelial / tubules

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14
Q

preop chemo regimen

A

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

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15
Q

post op histology risk stratification

A

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

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16
Q

mesoblastic nephroma

A

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

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17
Q

metanephric blastema

A

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

18
Q

nephroblastomatosis

A

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

19
Q

types of RMS

A

embryonal >50% - botryoid, spindle cell paratesticular

alveolar 20%

20
Q

investigations RMS

A

pelvic and urinary tract US

21
Q

histological components Wilms

A

metanephric blastema
primitive renal tubular epithelium
connective tissue components

22
Q

mutation wilms

A

chromosome 11 mutation or deletion WT1 tumour supressor gene

23
Q

Denys Drash syndrome

A

Wilms

ambiguous genitalia

24
Q

Beckwith Wiedemann syndrome

A

Macroglossia

24
Q

associations wilms tumour

A

Denys Drash
Beckwith Wiedemann
Horseshoe kidney

25
Q

Rhabdomyosarcoma

organs involved

A
bladder base
prostate
paratesticular
uterus
vagina
26
Q

Rhabdomyosarcoma association

A

Li Fraumeni syndrome p53 mutation

27
Q

Rhabdomyosarcoma better vs worse prognosis form

A

embryonal forms have better prognosis, of which bladder mostly embryonal

28
Q

Neuroblastoma organ

A

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

29
Q

Neuroblastoma median age diagnosis

A

2 years

30
Q

Neuroblastoma poor prognosis

A

deletion short arm chromosome 1

31
Q

Neuroblastoma scan

A

MIBG scan highly sensitivie

32
Q

differential abdominal mass / renal mass

A

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

33
Q

wilms 5 year survival

A

85% five year survival

if unilateral localised then prognosis in high 90s

34
Q

pain in Wilms tumour

incidence haematuria

A

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

35
Q

imaging vs biopsy

A

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

36
Q

what to look for on imaging with wilms

A

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

37
Q

staging Wilms

A

can met into lungs so need CT check

38
Q

bilateral Wilms tumour

A

stage 5

need to think of nephron

39
Q

syndromes associated with Wilms

A

hemihypertrophy
WAGR
Denys Drash
Beckwith Wiedemann

tend to be multifocal and bilateral

40
Q

chemotherapy Wilms

A

Always give neoadjuvant chemo

vincristine
actinomycin
doxorubicin added selected cases
give 6 weeks
shrinks tumours
reduced rate of spillage at surgery
41
Q

Wilms surgery

A

aim to sample LN at least 7