Childhood cancers

Question 1

What is Wilms’ tumour and what age group is typically affected?

Answer 1

Wilms’ tumour is a specific type of tumour affecting the kidney in children, typically under the age of 5 years.

Question 2

What should be considered in a child under the age of 5 years presenting with a mass in the abdomen?

Answer 2

Wilms’ tumour

Question 3

How might Wilms’ tumour present?

Answer 3

Child <5 presenting with mass in abdomen +

Abdominal pain

Haematuria

Lethargy

Fever

Hypertension

Weight loss

Question 4

How is WIlms’ tumour diagnosed?

Answer 4

Abdominal USS - INITIAL INVESTIGATION
Initial investigation is an ultrasound of the abdomen to visualise the kidneys.

STAGING - CT OR MRI
A CT or MRI scan can be used to stage the tumour.

DEFNITIVIE DIAGNOSIS - BIOPSY
Biopsy to identify the histology is required to make a definitive diagnosis.

Question 5

Wilms’ tumour - definitive diagnosis

Answer 5

Biopsy

Question 6

Wilms’ tumour - initial investigation?

Answer 6

Abdominal ultrasound

Question 7

Wilms’ tumour - management

Answer 7

NEPHERECTOMY - surgical excision of the tumour along with the affected kidney (nephrectomy).

Adjuvant treatment refers to treatment that is given after the initial management with surgery. This depends on the stage of the disease, the histology and whether it has spread. The main options are:

• Adjuvant chemotherapy
• Adjuvant radiotherapy

Question 8

What is the prognosis of Wilms’ tumour?

Answer 8

Early stage tumours with favourable histology hold a good chance of cure (up to 90%). Metastatic disease has a poorer prognosis.

Question 9

What causes retinoblastoma (which occurs in childhood)

Answer 9

Germ line mutations in the retinoblastoma gene (Rb) predispose to retinoblastoma which occurs in childhood.