Wilms Tumor (Renal CA) Flashcards

1
Q

Wilms Tumor

A
  • one of the most common primary neoplasms of younger children (~2-5 yrs)
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2
Q

associated congenital abnormalities

A
  • aniridia
  • hemihypertrophy
  • anomalies of GI system
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3
Q

aniridia

A

absence of iris

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4
Q

hemihypertrophy

A

enlargement of 1 side of face/body

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5
Q

chromosomal abnormalities

A

one Wilms Tumor gene (WT1 on Chr11)

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6
Q

WT1 gene

A

encodes transcription factor critical for normal kidney development

(~10-20% of all Wilms Tumors have mutated WT1)

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7
Q

tumor characteristics

A
  • sharply defined & encapsulated
  • occurs in any part of kiney
  • grows to any size
  • distorts kidney structure
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8
Q

how many stages

A

4

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9
Q

stage I

A

limited to kidney & can be excised w/ capsular surface intact

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10
Q

stage II

A

extends into renal capsule, can be excised

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11
Q

stage III

A

extension of tumor occurs but is confined to abdo

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12
Q

stage IV

A

metastasis (commonly to lung via blood)

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13
Q

mnfts

A
  • largely asympt abd mass
  • HTN
  • some children may have abd pain, vomiting, or gross hematuria
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14
Q

Dx

A

CT scan

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15
Q

Tx

A
  • Sx
  • chemo
  • radiation (sometimes)
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16
Q

5-yr survival rate

A

70% for all stages

89-96% for stages I-III