Malignancy

Question 1

Describe the epidemiology of leukaemia

Answer 1

Leukaemia is the most common form of childhood cancer (accounts for about a third of all cases)

Question 2

What is the most common type of leukaemia in children?

Answer 2

Acute lymphoblastic leukaemia (ALL)

Question 3

Describe the clinical presentation of ALL

Answer 3

• Peaks at 2-5 years of age
• In most cases, ALL presents insidiously over several weeks, e.g. malaise, anorexia
• Signs of bone marrow infiltration, e.g.: pallor, lethargy (anaemia); increased susceptibility to infection (neutropenia); bruising, bleeding, petechiae (thrombocytopenia)
• Other signs: hepatosplenomegaly; lymphadenopathy

Question 4

Describe the investigation of suspected ALL

Answer 4

• FBC: low Hb, low WCC, low platelets
• Blood film: blast cells
• Bone marrow biopsy is diagnostic

Question 5

Describe the management of ALL

Answer 5

• Anaemia: blood transfusion
• Thrombocytopenia: platelet transfusion
• Neutropenia: treat infection
• Chemotherapy

Question 6

Describe the epidemiology of brain tumours in children

Answer 6

Brain tumours are the second most common form of childhood cancer after leukaemia (account for about 25% cases)

Question 7

What is the most common type of brain tumour in children?

Answer 7

Astrocytoma

Question 8

Are brain tumours in children primary or secondary?

Answer 8

Brain tumours in children are almost always primary

Question 9

Describe the clinical presentation of brain tumours

Answer 9

• Raised ICP symptoms: headache, vomiting, seizures

- Focal neurology, e.g. visual problems, balance/co-ordination problems, behavioural change

Question 10

Describe the investigation of suspected brain tumour

Answer 10

MRI

Question 11

Describe the management of brain tumours

Answer 11

• First line: surgery with the aim of maximum resection

- Second line: chemo/radiotherapy

Question 12

What is Wilms tumour?

Answer 12

Wilms tumour (nephroblastoma) is a type of renal tumour

Question 13

Describe the presentation of Wilms tumour

Answer 13

• Painless abdominal mass

- Haematuria

Question 14

Describe the pathophysiology and aetiology of retinoblastoma

Answer 14

• Malignant tumour of retinal cells
• May affect one or both eyes
• Hereditary association: retinoblastoma susceptibility gene is on chromosome 13
• If there is a family history of retinoblastoma, children are screened regularly

Question 15

Describe the clinical features of retinoblastoma

Answer 15

White pupillary reflex replaces red reflex

Question 16

Describe the management of retinoblastoma

Answer 16

Chemotherapy, followed by laser treatment to the retina

Question 17

Survivors of retinoblastoma are at a higher risk of…

Answer 17

Second malignancy (especially sarcoma)

Question 18

Describe the epidemiology of bone tumours

Answer 18

• Uncommon before puberty

- Male predominance

Question 19

Give some examples of types of bone tumour

Answer 19

• Osteosarcoma

- Ewing’s sarcoma

Question 20

Describe the clinical features of bone tumours

Answer 20

• Persistent, localised bone pain (limbs are most commonly affected)
• Otherwise well

Question 21

Describe the management of bone tumours

Answer 21

• Chemotherapy, followed by surgery (amputation is avoided wherever possible)
• Radiotherapy may also be required, e.g. if surgical resection is incomplete