Nephroblastoma (Wilm’s Tumour)

Question 1

Define

Answer 1

Most common intra-abdominal tumour of childhood (2nd most common cancer of childhood after ALL)

<5yo (80%) – often 3yo

95% unilateral

1-2% familial /FHx

Question 2

Associations

Answer 2

• Beckwith-Wiedemann syndrome (specific body parts overgrow; usually presents at birth; islet cell hyperplasia)
• WAGR syndrome (Wilm’s tumour, Aniridia, Genitourinary malformations, (mental) Retardation)
• Hemihypertrophy
• 33% with a loss-of-function mutation in the WT1 gene on chromosome 11

Question 3

Symptoms

Answer 3

Commonly:

• Asymptomatic abdominal mass (MOST COMMON)
• Smooth and firm
• Painless haematuria

Less common:
* Abdominal pain
* Anorexia
* Anaemia (haemorrhage into mass)
*

DDx:

Faeces - most common cause of abdo mass - usually craggy, mobile and in lower abdo

Neuroblastoma - most arise in the abdo (adrenal gland or retroperitoneal symp ganglia) - causes discomfort and have FLAWS symp

Question 4

Investigations

Answer 4

nvestigations – avoid biopsy as it may worsen the condition:

Urine Dip

USS

CT/MRI

 

Staging 1-5:

1 = limited to kidney, completely excisable

2 = not limited to kidney, completely excisable

3 = not limited to kidney, not completely excisable

4 = spread beyond abdomen, haematogenous metastasis

5 = bilateral (each tumour graded separately)

Question 5

Managemenet

Answer 5

Management:

Nephrectomy + chemotherapy (± radiotherapy prior to surgery if advanced disease)

80% cure rate