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Wilms Tumor > Wilms diagnosis and treatment > Flashcards

Wilms diagnosis and treatment Flashcards

1
Q

DDx for abdominal mass in children

A 
Neuroblastoma
Wilms tumor
Lymphoma 
Rhabdomyosarcoma
Soft tissue sarcoma 
Splenomegaly
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2
Q

How is neuroblastoma different at presentation?

A

Neuroblastoma has:

  1. Calcifications (50-90%)
  2. HMA, VMA
  3. Age is usually < 1 years
  4. Tumors can cross midline (rare for WT)
  5. Tumor moves with respiration (rare for WT)
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3
Q

What imaging is needed at work up?

A
  1. Abdominal US
  2. CT with contrast and MRI of abdomen
  3. CT chest and if positive get CXR
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4
Q

What labs are needed at work up?

A
  1. CBC
  2. UA
  3. BMP
  4. LFTs
  5. HMA
  6. VMA
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5
Q

What other work up is needed for clear cell variant after surgery?

A
  1. MRI brain
  2. Bone scan
  3. BM bx
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6
Q

What other work up is needed for rhabdoid variant after surgery?

A
  1. MRI of brain (cerebellar or pineal PNET)
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7
Q

Is biopsy recommended before surgery?

A

No usually, because it increases the risk for tumor spillage and contamination of abdominal cavity.

Only biopsy when the tumor is found to be unresectable or bilateral

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8
Q

Stage IV or V

A

IV: Hematagenous mets or LN mets outside of the abdomen (lung, liver, bone, brain)

V: Bilateral wilms tumors (5-10%)

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9
Q

Stage I or II

A

I: confined to the kidney and completely resected
II: Beyond the kidney but completely resected. The invasion can include penetration of the renal capsule or invasion of the renal sinus vessels

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10
Q

Stage III

A
  1. STR
  2. Inoperable
  3. Positive margins
  4. Diffuse tumor spillage or local spillage
  5. Abdominal/pelvic nodes
  6. Peritoneal implants
  7. Biopsy
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11
Q

General treatment scheme for Stage III FH, no LOH?

A
  1. Surgery
  2. Flank RT: 10.8 Gy by POD 9
  3. VAD chemo x 24 weeks
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12
Q

What is VAD chemo?

A
  1. Vincristine
  2. Actinomycin
  3. Doxorubicin
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13
Q

Which patients do not get RT?

A

WT FH Stage I-II

WT Clear Cell: Stage I with central path review

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14
Q

Flank field set up

A
  1. AP/PA with 4-6 MV
  2. Preop tumor + kidney + 1 cm
  3. Flash laterally
  4. Cover entire VB when PTV overlaps
  5. If paraaortic nodes are positive, include bilateral PA nodal chain from T11 to L4
  6. If orginal tumor extended over to the contralateral kidney, the medial border should be 1 cm lateral to the VB edge
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15
Q

What is the field set up for whole abdomen RT?

A

Whole abdomen RT

  1. Superior border: 1 cm superior to diaphragm
  2. Flash laterally
  3. Inferior border: Bottom of obturator
  4. Block femoral heads
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16
Q

What do you do after surgery?

A

Local imaging (CT scan/MRI) do determine if there is gross residual

17
Q

What do you do if lung mets are visible only on CT?

A

Chemo only. WLI is not useful.

18
Q

What dose do you use with flank RT?

A

FH
No residual: 10.8 Gy at 1.8 Gy/fx
Gross residual: PTV 10.8 at 1.8 Gy/fx and boost to an additional 9 Gy at 1.8 Gy/fx to the gross disease. Total 19.8 Gy.

RTK or Diffuse Anaplasia
No residual: > 1 year old19.8 at 1.8 Gy/fx. If < 1 year, then 10.8 Gy at 1.8 Gy/fx.
Gross residual: Boost gross disease to an additional 9 Gy.

19
Q

What dose for whole abdomen RT?

A

10.5 Gy at 1.5 Gy/fx

20
Q

What dose for whole lung RT?

A

12 Gy at 1.5 Gy/fx if older than 1
Boost gross disease to an additional 7.5 Gy

If < 1 years old
10.5 Gy at 1.5 Gy.fx

21
Q

What do you do if lung metastases are seen on CXR?

A

Whole lung RT

22
Q

Kidney dose constraints?

A

Opposite kidney < 14.4 Gy

Opposite kidney partial: 50% < 19.6 Gy 14.4 Gy

23
Q

Liver constraint?

A

Whole liver < 23.4 Gy

24
Q

Median Age

A

3-4

2.5 for kids with bilateral tumors

25
Q

Unfavorable histologies

A
  1. Diffuse anaplasia
  2. Rhabdoid
  3. Clear cell
26
Q

Genetic studies to get at work up?

A

In FH, get LOG of 1p and 16 q because with these there is a poorer RFS

27
Q

What are the indications for whle abdomen RT?

A

Diffuse tumor spillage
Peritoneal tumor seeding
Ctyology + ascietes
Pre-op or intraperitoneal tumor rupture

28
Q

What do you do with bone mets?

A

25.2 at 1.8 Gy/fx

CTV = lesion + 3cm

29
Q

What do you do with liver mets?

A

Whole liver RT to 19.8 Gy at 1.8 Gy/fx

30
Q

What do you do with brain mets?

A

Whole brain RT: 20.6 Gy at 1.8 Gy/fx

Whole brain RT + Stereotactic boost: 21.6 Gy then a 10.8 Gy boost

31
Q

What are the whole lung field borders?

A

Superior: SCL fossa bilaterally
Inferior: Posterior inferior aspect of diaphragm which is usually L1
Laterally: 1-4 cm lateral to ribs

32
Q

What addtional treatment do patients with WLI need?

A

PCP prophylaxis with Bactrim

33
Q

When do you reevaluate for a boost after WLI

A

2 weeks afterwards with a CT scan

34
Q

General treatment scheme for Stage III FH, with LOH?

A
  1. Surgery
  2. Flank RT: 10.8 Gy by POD 9
  3. VAD/CE chemo x 24 weeks
35
Q

When do you treat metastases?

A

At the conclusion of chemotherapy

36
Q

Risk of second cancers

A

2%

37
Q

Risk of SBO

A

15% at 15 years

38
Q

Beck width-Weismann

A

Macroglossia, macrosomia, omphalmocele, hemihypertrophy

Wilms Tumor (1 decks)

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