Wilms Tumor Flashcards

1
Q

Wilms tumor is the commonest primary renal tumor of childhood T/F?

A

True

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2
Q

Age ranges for wilms tumor?

A

1-5 years
Peak age- between 3-4 years

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3
Q

Median age of presentation for unilateral and bilateral respectively?

A

Unilateral- 3yrs , 6 months
Bilateral- 2 years , 6 months

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4
Q

Sporadic: familial ratio

A

99% : 1 %

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5
Q

Symptoms of wilms tumor?

A

Hematuria
Weight loss
Obstipation
Diarrhea
Urinary Tract Infection
Previous trauma
Nausea
Abdominal pain

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6
Q

Signs of Wilms tumor?

A

Palpable mass in abdomen( flank mass which doesn’t cross the midline.
Hypertension due to hyper reninemia

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7
Q

What congenital anomalies are associated with wilms tumor?

A
  1. WAGR syndrome/ 11p deletion syndrome: Wilms tumor, Aniridia, Genitourinary anomalies and Mental Retardation.
  2. Denys- drash syndrome- Wilms tumor, early renal failure with mesangial sclerosis and pseudo hermaphroditism.
  3. Beckwith- wiedemann syndrome-
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8
Q

What are the associated paraneoplastic syndromes

A

Thrombocytosis
Bleeding diathesis due to presence of acquired von Willebrand disease.
Hypertension
Erythropoietin increased: males , older age and low clinical stage

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9
Q

What are the local patterns of spread?

A

Renal sinus or ureter
Renal vein into IVc and rarely, right atrium.
Regional lymph node involvement

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10
Q

Hematogenous spread patterns

A

Lungs- 80%
Liver- 15%
Rarely bone, bone marrow or brain

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11
Q

WT stage I

A

Confined to kidney, intact capsule

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12
Q

WT stage II

A

One kidney with breeched capsule

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13
Q

WT stage III

A

Residual tumor( when biopsy is done)

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14
Q

WT stage IV

A

Metastatic disease

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15
Q

WT stage V

A

Bilateral disease

Stage each kidney separately

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16
Q

What Investigations will I do in WT?

A

History- family history of cancer, congenital defects
P/e- congenital abnormalities
FBC
Urinalysis and Renal function test
LDH, Uric acid and clotting screen
Echocardiogram
Abdominal USG/CT/MRI
Chest X-ray or CT

17
Q

Treatment options?

A

Neoadjuvant / Adjuvant chemotherapy
💊stage I or II: vincristine + Dactinomycin
💊 stage III or IV: add doxorubicin + renal bed radiotherapy
☢️radiotherapy for lung metastasis
😷Surgery (primary or delayed) for all patients