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Oncology HW > Wilms > Flashcards

Wilms Flashcards

1
Q

3 most common syndromes associated with WT

A

10 % of WT

WAGR
-Wilms, aniridia, genitourinary malformation, mental retardation

Li-Fraumeni (tp 53 - anaplastic)

Beckwith-wiedemann (overgrowth syndrome)
-visceromegaly, macroglossia, hyperinsulinemia and hypoglycemia, omphalocele, wilms, hepatoblastoma

Denys-drash
-wilms, nephropathy and renal failure, male pseudohermaphrodism

Also…
hemihyperthrophy, isolated aniridia

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2
Q

incidence of WT

A

7.6 case per 1 million

more frequent amongst african american children

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3
Q

mean age at diagnostic

A

36 months

Incidence decreases significantly after 10 years of age. also, WT uncommon < 6 months old.

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4
Q

Name 3 genes associated to WT

A

tp 53: anaplastic histology (Li-Fraumeni)
WT 1
WT 2

LOH at 1p & 16q is also important and has a prognostic impact.

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5
Q

What is a nephrogenic rest?

A

persistent metanephros blastema (embryonal kidney tissue) beyond 36 week.

thought to be a precursor or WT.
Hyperplastic NR cannot be differenciated from WT on needle bx: needs capsule.

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6
Q

What is nephroblastomatosis?

A

presence of multiple or diffuse neprogenic rests. Risk factor for WT (metachrone contralateral WT)

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7
Q

What is WT rule of 10s?

A
  • 10% bilateral
  • 10% syndromic
  • 10%unfavorable histology
  • 10% have tumoral venous extension
  • rare after 10 yo
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8
Q

Describe WT histology

A

Favorable 90%
-mix of blastemal, stromal, epithelial tubules. can be tri/bi/monophasic with no difference in outcome

Unfavorable 10% (TP53 chromosome depletion)
-focal or diffuse anaplasia

One of these criteria is required for diffuse :

  • presence of anaplasia in random kidney bx
  • anaplastic cells outside the kidney
  • anaplastic cells in more than 1 region of the kidney
  • extreme pleomorphism
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9
Q

What are the principle management differences between SIOP and COG?

A

SIOP treats WT upfront with neoadjuvant chemo, regardless of resectability. advantage: decreases size of tumor and decrease rate of intraop tumor rupture

COG focuses on nodal staging and chemo sparing. emphasis on importance of nodal staging + avoidance of biopsy to reduce more intensive chemotherapy. increase tumor rupture noted compare to SIOP

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10
Q

Renal tumor treatment is centered on risk stratification according to which factors?

A 
Histology
local and disease stage 
age
tumor weight
response to therapy
biogical marker such as LOH
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11
Q

Which group can be spared adjuvant chemotherapy?

A

< 2 yo
tumor weight < 550 g
stage 1
favorable histology

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12
Q

Surgical principle at time of nephrectomy?

A
  • generous transperitoneal incision
  • Complete abdominal exploration (implants, nodes, hemoperitoneum)
  • No need to palpate/explore contralateral kidney (CT surfficient)
  • palpate IVC / renal vein / ureter for invasion
  • ligate/divide ureter as low as possible
  • preserve gerota’s facia
  • remove adrenal if upper pole tumor. ok to preserve otherwise
  • attempt vascular control first but abort if dangerous
  • prevent tumor rupture and spillage
  • no in vivo bx except if unresectable
  • if vascular extent, remove with specimen (do not transect)
  • preserve surrounding organs
  • lymph node sampling (5 or+)
  • clips where margin +
  • send fresh
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13
Q

What is the management of stage V cancer

A

Stage 5: bilateral tumor

  • Initial biopsy not recommended. if clinical picture unclear (eg > 10yo) and bx is preferable, perform a posterior needle biopsy
  • Start with 3 drug chemo (Vincristine, actinomycine, doxorubicin).
  • reassess resectability at 6 weeks.
  • if <50% in size, perform bilateral biopsy or perform nephron sparing nephrectomy if possible
  • If good response (>50%) but not resectable, continue another 6 weeks of chemo.
  • Operate at 12 weeks. Nephrectomy only if kidney not salvagable
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14
Q

What are the contraindications for upfront nephrectomy?

A

COG: remove kidney + LN sampling upfront, except if:
1) bilateral tumor
2) would require multivisceral resection
3) Single kidney
4) overwhelming lung metastatic burden and too
sick for OR
5) extension of venous thrombus to hepatic veins
6) unilateral tumor in patient with WT associated syndrome (nephron sparing)

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15
Q

How is WT staged?

A

Abdominal (local) staging and disease staging

Local staging:
1- R0 N0, no capsular invasion, no tumor in renal sinus,

2- R0 N0 but: capsular penatration or vascular invasion (invludes sinus, venous thrombus)

3-R1 N0 / R0 N+ / R2 . include preop biopsy, pre/intra op rupture (includes peritoneal implants)

4-M+ (include extraabdo + LN)

5- bilateral tumor

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16
Q

What are the indications for nephron sparing surgery?

A

1) unilateral kidney
2) bilateral tumor
3) tumor in context of WT associated syndrome (denys-drash, bekwith-wiedemann, WAGR)

17
Q

What is the consequence of a preop bx or intraop tumor rupture?

A

patient goes from a 2 to 3 drug regime (VA -> VAD) + requires at least flank radiotherapy (10 Gy)

18
Q

When do patient receive radiotherapy?

A

All patient with soilage requires flank radiotherapy (10 Gy).

Total abdominal radiotherapy given only if:

1) preop tumor rupture
2) peritoneal implants
3) gross intraop tumor spill

Isolated lymph node involvement does not represent an indication for total abdo rxtx.

19
Q

What is the management of venous thrombus extending to atrium?

A

Image with CT + echo
neoadjuvant treatment VAD
reassess at 6 weeks
If persistent of atrial extent, give another 6 weeks of chemo
if persistent, perform surgery with CVT and cardiopulm bypass
Expose retrohepatic vena cava + venotomy with concomitant atriotomy.
remove thrombus in concert with CVT to avoid embolisation

20
Q

what is the management of lung mets.

A
  • WT lung mets are peripheral + superficial
  • 1/3 of lung nodules <1 cm are not mets.
  • thoracoscopy acceptable

Answer depends on clinical presentation:
-if patient has local stage 1-2 and has a few lung nodules (unilateral), perform wedge resections to prove mets as an upstaging will require the addition of doxorubicine to V+A. For local stage 3-4, will get VAD anyways, so no need to bx lungs.

-after 6 weeks re-image.
if mets melted (40%), continue chemo for a total of 25 weeks (VAD)
if mets persist and numerous, escalate to 5 agent chemo (VAD, cyclophosphamide and etoposide) + lung rad.

recently, if only a few nodules in 1 lung, can attempt resection: if demonstration of tumor necrosis, may avoid escalating to 5 drug chemo/lung radiation.

If lung mets persists after completion of chemo/rad, may consider resection to render tumor free.

21
Q

What is the intraop management for ureter tumoral extension?

A

Palpate ureter to feel for tumoral extension. If evidence of tumor, transect distal to thrombus, as low as possible.

22
Q

How to manage horseshoe kidney?

A

Perform preop renal scintigraphy to ensure both kidneys are functional. if so,
treat as a unilateral tumor.
at time of resection, be careful as frequent vascular anomalies + variation in ureteral implantation site.
resect isthmus with neprectomy specimen and perform standard LN sampling.

23
Q

How is a nephron sparing surgery performed?

A
  • Same approach as radical.
  • May use intraop US.
  • If possible, don’t transect/bx tumor.
  • obtain vascular control
  • transect kidney with energy device / mono or unipolar.
  • 0.5-1cm margin.
  • close collecting system with resorbable monofilament.
24
Q

What is the indication of a radical nephrectomy in stage V ?

A
  • nephron sparing not feasible after 12 weeks of chemo
  • syndromic WT with unilateral tumor: if < 50% response after 6 weeks (likely not chemo responsive), or if nephron sparing not feasible after 12 weeks,
25
Q

What is the classical clinical presentation of WT?

A

Asx toddler (average age at dx 3yo) with abdominal mass.

May present following tumor hemorrhage after minor trauma.

Rare: L gonadic vein thrombus extension with varicocele.

May present with hypertension (not often)

26
Q

What are radiological finding on CT

A

Lung mets: peripheral and superficial

Abdo:

  • claw sign of kidney
  • vessels pushed, not encased as in NB
  • Venous tumor extention
  • solid/cystic component due to hemorrhage
27
Q

What are you looking for on abdo CT for tumor staging

A
  • presence of 2 kidneys
  • bilateral disease
  • vascular extention of tumor
  • ureteral tumor extension
28
Q

What is the metastatic workup

A

CT Chest, abdo, pelvis

CBC, LFT, Coags (8% Von willebrand disease)

heart echo if suspect atrial thrombus

29
Q

When is radiotherapy given?

A

10-14 days postop.

decrease in survival if surgery to abdo radiotherapy interval > 14 days…

30
Q

What are the typical renal tumors per age groups?

A

0-6 months: mesoblastic nephromas

1-4 yo: WT. renal cell carcinoma

teenages: clear cell carcinomas., renal cell carcinoma

31
Q

What is the lifetime isk of ESRD post nephrectomy for WT?

A

0.6%

32
Q

Metastasis site?

A

Lung
liver
Brain

Oncology HW (11 decks)

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