Wilms tumour

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Introduction

Wilms tumour (nephroblastoma) is a malignant embryonal tumour of renal tissue.

About 80% of children with Wilms tumour present before 5 years of age. It can present soon after delivery.

It may be associated with congenital anomalies such as hemihypertrophy and the absence of the iris (aniridia).

Wilms tumour has a very good prognosis with an over 80% chance of long-term cure.

Causes of Wilms tumour

  • Sporadic gene mutation.

Symptoms of Wilms tumour

The symptoms of Wilms tumour include the following:

  1. Visible and or palpable abdominal mass
  2. Fever
  3. Blood in urine

Signs of Wilms tumour

The signs of nephroblastoma include the following:

  1. Abdominal mass (palpate with care to prevent dissemination or rupture)
  2. Haematuria (macroscopic or microscopic)
  3. Hypertension
  4. Associated congenital anomalies

Investigations

  • Abdominal ultrasound scan
  • Abdominal CT scan
  • Chest X-ray
  • Full Blood Count
  • Blood Urea, electrolytes and creatinine

Treatment for Wilms tumour

Objectives

The treatment objectives of Wilms tumour are

  1. To obtain long term cure
  2. To provide adequate supportive and palliative care

Non-pharmacological treatment

  1. Nephrectomy
  2. Radiotherapy post-surgery for advanced cases

Pharmacological treatment

A. Pre-operative treatment

1st Line Treatment

Evidence Rating: [A]

Vincristine, IV,

And

Actinomycin D, IV,

And

Doxorubicin, IV, (if presence of metastases)

B. Post-operative treatment

Evidence Rating: [A]

  • Vincristine, IV,

And

  • Actinomycin D, IV,

And

  • Doxorubicin, IV, (depending on stage and risk category)

Or

  • Carboplatin, IV,
  • Etoposide, IV,
  • Doxorubicin, IV, Cyclophosphamide IV in combination (For high risk tumours)

C. For treatment of vomiting

Evidence Rating: [A]

Metoclopramide, IV or oral,

  • 100-400 microgram/kg 8 hourly

Or

Granisetron, IV,

  • 40 microgram/kg (max. 3 mg) stat.

May repeat 12 hourly if necessary
Granisetron, oral,

  • 20 microgram/kg (max. 1 mg) within 1 hour before start of treatment
  • Then 20 microgram/kg 12 hourly for up to 5 days

Or

Ondansetron, IV,

Adults

  • 5 mg/m2 stat.
  • Repeat 8 hourly if necessary

Children

  • 12-18 years; 8 mg stat. (immediately before chemotherapy)

Or

Ondansetron, oral,

Adults: 8 mg 8 hourly, administered 30 minutes before the start of chemotherapy

Children:

  • 12-18 years; 8 mg 8-12 hourly up to 5 days
  • 1-12 years; 4 mg 8-12 hourly up to 5 days

D. For treatment of febrile neutropenia

Ceftriaxone, IV, 100 mg/kg daily

And

Gentamicin, IV, 5 mg/kg daily.

And

(if still febrile after 48 hours, add)
Cloxacillin, IV, 25-50 mg/kg 6 hourly

And

 (if still febrile after 5 days, add)
Fluconazole, oral, 10 mg/kg daily

E. For treatment of anaemia and thrombocytopenia

Blood and blood product transfusions
(See ‘Bleeding disorders‘)

Referral Criteria

All patients should be referred to specialist centres for appropriate treatment.

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