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Haem/Onc > Wilms Tumour, ASPHO > Flashcards

Wilms Tumour, ASPHO Flashcards

1
Q

give 4 peds renal tumours other than wilms

A 
RCC
malignant rhabdoid
clear cell sarcoma of kidney
congenital mesoblastic nephroma
cystic nephhoma
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2
Q

neprhogenic rests

A

= persistentce of foci of embryonic renal tissue after normal nephrogenesis

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3
Q

which neprhogenic rests are associated with increased risk fo progression to wilms? associated with waht mutations?

A

intralobar

Wt1

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4
Q

which neprhogenic rests are associatd with sporadic tumours? associated with what else?

A

perilobar;
BWS
hemihypertrophy
dysregulation at 11 p15

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5
Q

neprhoblastomatosis=? associated with?

A

multilobar or diffuse nephrogenic rests; high risk of developing WT

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6
Q

muts taht are most frequent in wilms? 3

A

WT1
WT2
WTX

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7
Q

where is WT1 located? function?

A

11p13; trx factor, tumor suppressor

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8
Q

WT1 mutation seen in which syndromes?

A

WAGR
Denys-Drash
Frasier syndrome

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9
Q

features seen in WT1 mutations?

A
  • bilateral wilms
  • unilateral wilms with nephrogenic rests at contralateral kidney
  • stromal and rhabdomyomatous differentation
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10
Q

WT2 associated syndromes?

A

Beckwith-Wiedemann

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11
Q

other than WT1/2/X, 2 other genes that can be associated with wilms?

A

DICER1, TP53

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12
Q

TP53 found in what type of wilms?

A

anaplastic

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13
Q

5 features of BWS?

A 
exomphalus
*omphalocele
*macroglossia
*macrosomia
*neonatal hypoglycemia
*viscerogmegaly
*hemihyperplasia
cancer predispo
high IGF2 expression
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14
Q

WAGR features?

A

wilms, aniridia, GU abN, range of intellectual disability

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15
Q

Denys-drash features?

A

progressive renal disease, pseudohermaphrodism

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16
Q

fraiser syndrome features?

A

focal segmental glomerulosclerosis, male gonadal dysgenesis

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17
Q

other than BWS, wagr, frasier, denys-drash, 2 others asscoiated with wt?

A 
perlman
bohring-opitz syndrome
fanconi anemia
dicer1
LFS
familial WT
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18
Q

wilms affects M or F more?

A

F

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19
Q

median prseentation for WT?

A

3

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20
Q

% of WT= favourable histo?

A

95%, vs. 5% anaplastic

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21
Q

Risk strat factors for wilms? 5

A
  • histo
  • stage
  • age
  • tumour wt
  • tumour genetic (LOH 1p, LOH 16q, gain 1q, 11p15 LOH)
  • CPS
  • resolution of pulm lesions
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22
Q

triphasic components of favourable histo in Wilms?

A
  • epithelial
  • blastemal
  • stromal
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23
Q

___ predominant often seen with ___ risk pathology

A

blastemal; higher

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24
Q

SIOP psot chemo histo risk assessmetn in wilms?

A

-LR: completely necrotic tumour, cystic partially differenaited tumour
IR: epitehlail, stroma, mixed or regressive type, focal anaplasia
HR: bastemal predominant, diffuse anaplasia

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25
Q

Describe stage 1 wilms

A

-tumour limited to kidney and completely resected, capsule in tact, LNs are neg

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26
Q

stage 2 wilms?

A

confined to renal fossa, LNs are neg, cmpletely resected, can ext only into renal sinus/capsule

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27
Q

stage 3 wilms?

A

microscopic or gross residual disease in abdo after surgery (Non-hematogenous spread)…also if tumour removed if more than one piece (eg tumour thrombus in renal vein removed separately)

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28
Q

stage 4 wilms?

A

hematogenous sprad to lungs, liver, bone, brain…or LNs outside of abdo

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29
Q

stage 5 wilms?

A

bilateral, 5-10%

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30
Q

wilms pres?

A
  • abdo mass
  • abdo pain
  • hematuria
  • htn
  • acquired vwd
  • fever
  • anemia
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31
Q

work up for wt?

A

US
CT/MRI
CT chest

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32
Q

feature seen on abdo imaging in WT?

A

“claw sign”= normal kidney surrounding tumour

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33
Q

survival rate in wt?

A

> 90%

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34
Q

COG advocates for upfront nephrectomy whenever possible: PROS of this?

A

BR HS…“BRian mcmahon High School”

  • immediate removal of bulk disease
  • establish histo dx and appropr therapy
  • full upfront tumour staging
  • use of tumour bio to guide therapy
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35
Q

if cannot do upfront nephrectomy, do what?

A

BIOPSY! core or open needle…NOT fine needle

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36
Q

SIOP recs neoadj chemo instead, why?

A

based on premise taht vast majority of peds renal tumours= WT adn all localzied tumours get VA (vs. VAD for met disease)

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37
Q

advantages of neoadj chemo in wilms?

A

“RCT”

  • lower surgical Complication rate
  • less Rupture, could –> downstaging
  • ability to base Therapy off response to chemo with post-chemo histo risk stratification system
38
Q

does SIOP advocate for bx?

A

NO…due to risk of tumour spread thru biopsy

39
Q

SIOP recs upfront nephrectomy for which population?

A

<1 year and >10 yrs of age…less likely to have FH wilms

40
Q

Very LR criteria in AREN0532?

A

stage 1, FH WT
neg LNs
tumour wt <550 g
pt < 2 yrs

…can’t have CPS, multicentric tumour or contralateral Nephrogenic rests

41
Q

very low risk WT: manage?

A

obsrvation alone unless LOH 11 p15, LOI 11p15 (these pts relapsed)–> EFS 89.7%, 100% OS

42
Q

AREN0532: what happened to relapsed cases?

A
  • relapsed EARLY (4 months)

- got DD4A and radiation, which worked well

43
Q

conclusion of AREN0532?

A

observation after surgery is recommended for Very low risk….but EE4A with vcr and actinomycin= reasonable alternative

44
Q

which pts are LR wilms?

A

stage 1 adn 2 wtih favourable histo…can’t have LOH at 1 p or 16q

45
Q

EFS and OS of LR wilms?

A

OS 98.4%, EFS 91.4%

46
Q

standard of care for LR wilms pts?

A

EE4A: 19 weeks x vcr, actinomycin after upfront necphrectomy

47
Q

stage 3 pts treated how on AREN0532?

A

DD4A= 25 weeks x vcr, actinomycin, doxorubicin with flank/whole abdo rads…this study removed those with LOH at 1p, 16 ro anaplasia…EFS 88%, OS 97%

48
Q

if your LNs are negative adn no LOH, what happens?

A

better survival

49
Q

factors associated with relapse in WT?

A

+ LNs, LOH at 1p or 16q

50
Q

stage 4 tx for WT as per AREN0533?

A

if lung disease: Vcr, Actino, Doxo…assessment at 6 weeks–> complete response: continue…if incomplete: moved to Remigen M with cyclo/etop and whole lung XRT

51
Q

AREN0533 outcomes for stage 4 wilms with slow response for pulm nodules?

A

EFS 88.5%, OS 95.4% (much better than DD4a with lung rads)…regimen M = best for pateints iwth slow incomplete response of pulmonary lesions…associated with increased tox though adn poor salvage after relapse

52
Q

AREN0533: outcomes for stage 4 with complete response to pulm nodules?

A

got DD4a, NO lung rads–> EFS 79.5%, OS 96.1%…recurrences mostly in the lung…similar results to NWTS with lung rad results….allowed many pateints to be spared from rads! however, those who had 1q gain did MUCH worse when they looked at that…a reduction of therapy (no lung RT) is not recommended for those with 1q gain

53
Q

wt stage 4: slow incomplete response and/or 1q gain: give what?

A

regimen M

54
Q

WT stage 4 with mets OTHER than lung: DD4A x 6 weeks–> regimen M, rads to all met sites: outcomes?

A

no improvvement vs those who just got dd4a on NWTS…needs mroe studying

55
Q

pts with LOH 1p AND 16q…if combined, they had what?

A

markedly lower EFS

56
Q

ARENO532 and 33 offered what?

A

offered intesifcation of therapy…for stage 1-2, vcr, actino + DOXO….for 3-4: got regimen 4= vcr, actino, doxo + 4x Cyclo/etop, plus rad

57
Q

intensifing thearpy for Stage 1-2 with LOH 1p and 16q on AREN0533 did what?

A

increased EFS and OS but not signif

58
Q

intensifing thearpy for Stage 3-4 with LOH 1p and 16q on AREN0533 did what?

A

improved EFS adn OS, was signficant

59
Q

conclusion for LOH 1p + 16q:

A

for stage 3-4, intesnification to regimen M should be standard of care

60
Q

anaplastic WT associated with?

A

higher stage

older age

61
Q

anaplasia in WT can be focal or diffuse. with intesnifiation or regimens, OS has __ __

A

trended up (but is still low, esp for stage 4)

62
Q

most common reanl tumour in adults?

A

rcc

63
Q

most common renal tumour in kids>15?

A

RCC

64
Q

RCC management?

A

surgery= most affective! need LN dissection.

cheo, rads don’t work too well

65
Q

RCC management?

A

-surgery= most affective! need LN dissection.
chemo, rads don’t work too well
-systemic: VEGF tyrosine kinase inhibitor (AXITINIB), mTOR inhbiition, anti-PDL1 antibodies (NIVOLUMAB)

66
Q

mean age pres rCC?

A

12 yrs

67
Q

most common histo subtype of RCC?

A

TFE3 or TFEB translocation associated

68
Q

CPS associated with RCC?

A
  • von Hippel-Lindau
  • tuberous sclerosis
  • familial RCC
  • herediatary leiomyomatosis and RCC
69
Q

vHL inheritance?

A

AD

70
Q

renal medullary carcinoma seen in which pts? histo?

A

sickle cell trait…loss of INI-1= characteristic…very aggressive tumour with poor prog

71
Q

most common sutbype of RCC?

A
  • translocation-associated RCC, accountsfor 1/2 of RCC in kids
  • Xp11.2 translcation, strong nuclear exprsesion of TFE3 or TFEb
72
Q

clear cell sarcoma of kidney: tends to met or no?

A

tends to met! bone, brain, soft tissue

73
Q

clear cell sarcoma of kidney : age? molec findings? gender?

A
  • median age: 3
  • BCOR ITD’s in 80%!
  • more in males
74
Q

AREN0321: tx for CCS of the kidney?

A

stage 1: regimen I= vcr, doxo, cyclo, etop…NO rads
stage 2-3: same as 1, with rads
stage 4: UH-1 therapy: vcr, doxo, cyclophos, etop

75
Q

what about relapse in CCSK?

A

typically LATE relapses, including to bone and brain

76
Q

malignant rhabdoid tumour of the kidney seen in what age?

A

<1 year mostly

77
Q

malignant rhabdoid tumour of the kidney associated with what on histo?

A

lack of INI1 expression!

78
Q

mets in malig rhabdoid tumour?

A

yes, often to lungs, abdo, LNs, liver, bone brain at dx

79
Q

malig rhaboid tumour can also presentat same time with what?

A

AT/RT

80
Q

prog of malignant rhabdoid tumour?

A

very poor

81
Q

tx malig rhabdoid tumour how as per AREN0321?

A

vcr/irinotecan didn’t work–> UH-1 with VDC/CCyE

vcr, doxo, cyclophos, carboplat, etop

82
Q

congenital mesoblastic nephroma: almost exclusively detected when?

A

first year of life

83
Q

most prevalent renal tumour in first 3 months of life?

A

congenital mesoblastic nephroma

84
Q

clinical pres in CMN?

A

htn
elevated ca (can continue after resection)
elevated renin

85
Q

CMN: histo can be classic and cellular and mixed…cellular type associated with?

A

t(12;15)= ETV6-NTRK3..which is also found in infantile fibrosarcoma

86
Q

CMN outcomes?

A

excellent with surgery only.

87
Q

what drug is being considered for metastatic CMN with 12;15 transloc

A

larotrectinib (TRK inhibitor)

88
Q

cystic nephroma presents when?

A

2.3 years

89
Q

cystic neprhoma associated with what muts?

A

DICER1! sent to Cancer gen!

90
Q

cystic nephroma: tx how?

A

surgery only

Haem/Onc (30 decks)

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