Paediatric cancers

Question 1

Retinoblastoma : Definition

Answer 1

1. Cancer which develops from cells in the retina which is responsible for vision
2. Most common ocular malignancy in children

Question 2

Retinoblastoma : Incidence

Answer 2

Occurs in children < 5 years
Average age of diagnosis : 18 months

Question 3

Retinoblastoma : Pathophysiology

Answer 3

1. Autosomal dominant disease
   * Heridatory mutation of retinoblastoma tumour suppressor gene which normally inhibits uncontrolled cell growth

Question 4

Retinoblastoma : Clinical features

Answer 4

1. Leukocorcia : red-reflex is lost, white glow in affected eye noticed in flash photographs
2. Strabismus
3. Decrease in vision
4. Redness/pain in the eyes

Question 5

Retinoblastoma : Management

Answer 5

1. Radiation/Chemotherapy
2. Enucleation : removal of the eye

Question 6

Retinoblastoma : Prognosis

Answer 6

Excellent > 90% surviving to adulthood

Question 7

Wilm’s tumor : Definition

Answer 7

Kidney cancer that primarily affects children
Most common childhood cancer

Question 8

Wilm’s tumor : Pathophysiology

Answer 8

1/3 due to genetic factors
* Inherited mutation of WT1 (Wilms tumor 1) which is a tumor suppressor gene in the kidneys

Question 9

Wilm’s tumor : Incidence

Answer 9

• Average age of diagnosis : 3 years
• Typically presents before 5 years

Question 10

Wilm’s tumor : Clinical feature

Answer 10

1. Painless abdominal mass - most common
2. Painless haematuria
3. Flank pain

Question 11

Wilm’s tumor : Management

Answer 11

1. Refer to paeds within 48 hours
2. Nephrectomy, Chemotherapy

Question 12

Wilm’s tumor : Prognosis

Answer 12

80% cure rate