Nephroblastoma (Wilms’ Tumour)
Description
Nephroblastoma (Wilms’ tumour) is the commonest malignant renal tumour of childhood, peaking between 2–5 years.
It is an embryonal neoplasm of kidney cells, arising unilaterally in >90% or bilaterally in a minority.
Occurs equally in boys and girls, and may be associated with congenital anomalies and syndromes.
A child presenting with an abdominal mass should be referred immediately for imaging.
If a renal mass suspicious for Wilms’ tumour is confirmed, urgent referral to a paediatric surgeon/urologist for biopsy or removal of the mass is required.
Needle biopsy is generally avoided, except in unusual clinical or imaging scenarios, due to high risk of tumour spillage and stage up-classification.
Signs and Symptoms
Nephroblastoma commonly presents as a painless abdominal mass.
Other symptoms and signs:
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Hypertension
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Haematuria (gross or microscopic, 12–25%)
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Constipation
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Weight loss
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Dysuria
Less common features:
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Nausea/vomiting
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Abdominal pain
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Cardiac insufficiency
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Pleural effusion
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Polycythaemia
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Hydrocephalus
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Acquired von Willebrand disease
Investigations
Laboratory Tests
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Full blood count (FBC)
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Urea, electrolytes, creatinine (U/E + Cr)
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Liver function tests (LFTs)
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Coagulation profile
Cardiac Assessment
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ECG
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Echocardiogram
Imaging
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Abdominal Ultrasound
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IVU
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Chest X-ray
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Contrast-enhanced CT scan of chest, abdomen, pelvis
Biopsy
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Needle biopsy is not advised, except in atypical or unclear cases.
Treatment
Management is multimodal, risk-stratified using surgery, chemotherapy, and radiotherapy.
Pre-operative Chemotherapy (Neoadjuvant)
Used where upfront surgery is not possible (inoperable tumours).
Localised Disease – VA Regimen
| Drug | Week 1 | Week 2 | Week 3 | Week 4 | Notes |
|---|---|---|---|---|---|
| Vincristine 1.5mg/m² IV push (0.05mg/kg if <10kg/BSA <0.6m²) | * | * | * | * | Reassess radiologically and consult surgery |
| Actinomycin D 0.05mg/kg IV push | * | – | * | – | Adjust dose for infants |
Bilateral Disease – VA Regimen
| Drug | W1 | W2 | W3 | W4 | Stop/Reassess | W5 | W6 | W7 | W8 | W9 | W10 | W11 | W12 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Vincristine | * | * | * | * | If good response → continue | * | * | * | * | – | – | – | – |
| Actinomycin D | * | – | * | – | Continue if good response | * | – | * | – | – | – | – | – |
Metastatic / Bilateral Disease – VAD Regimen
| Drug | W1 | W2 | W3 | W4 | CXR | W5 | W6 | W7 | W8 | W9 |
|---|---|---|---|---|---|---|---|---|---|---|
| Vincristine | * | * | * | * | If no mets → surgery | * | * | – | – | – |
| Actinomycin D | * | – | * | – | If mets → 3 more cycles | * | – | * | – | – |
| Doxorubicin 50mg/m² IV | * | – | – | * | – | * | – | – | – |
Surgery
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Radical Nephrectomy + Lymph Node Sampling
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Nephron-sparing surgery for bilateral tumours
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Performed upfront if operable, or after neoadjuvant therapy
Radiotherapy
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Indicated in select risk groups
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Should be initiated 2–6 weeks post-nephrectomy
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Requires referral to a centre with radiotherapy capability
Post-surgery Adjuvant Treatment
Depends on risk stratification (SIOP/COG guidelines).
Ensures optimal survival and reduction in relapse risk.