Oncology

Question 1

Define the following brain tumours:
1. Astrocytomas
2. Meningiomas
3. Ependymomas
4. Craniopharyngiomas

Answer 1

1. Tumour in astrocytes (star-shaped glial cells that perform many functions in the central nervous system (CNS))
2. Tumour in the meninges of the brain
3. Tumour in the cells of the ventricular system - commonly in posterior fossa and spinal cord
4. Tumour near the pituitary gland and hypothalamus

Question 2

1. What is the most common malignant brain tumour in children?
2. Where does it typically arise?
3. What are it’s symptoms?

Answer 2

1. Medulloblastoma
2. In cerebellum
3. Headache, vomiting, ataxia and cranial nerve deficits

Question 3

1. What is the most common benign brain tumour in children?
2. Where is it found?
3. What are it’s symptoms?

Answer 3

1. Pilocytic Astrocytoma
2. In the cerebellum or optic pathway
3. Headache, nausea, visual disturbance and balance issues

Question 4

Ix for brain tumours?

Answer 4

• MRI/CT brain
• LP - CSF examination
• Biopsy

Question 5

What is Wilms’ tumour?

Answer 5

A specific type of tumour affecting the kidney in children, typically under the age of 5 years.

Question 6

Sx of Wilms tumour?

Answer 6

• Abdo mass and pain
• Hameturia
• Lethargy
• Fever
• HTN
• Wgt loss

Question 7

Ix of Wilms tumour?

Answer 7

1st line - abdo USS
CT/MRI - stage tumour
Biopsy - histology

Question 8

Mx of Wilms tumour?

Answer 8

• Surgical excision - nephrectomy
• Chemo and radiotherapy

Question 9

What is neuroblastoma?

Answer 9

The tumour arises from neural crest tissue of adrenal medulla (MC site) and sympathetic nervous system.

Question 10

Sx of neuroblastoma?

Answer 10

• Abdo mass
• Pallor, wgt loss
• Bone pain, limp
• Hepatomegaly
• Paraplegia
• Proptosis

Question 11

Ix for neuroblastoma?

Answer 11

Abdo x-ray - calcification
Biopsy
Raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels

Question 12

What is retinoblastoma?

Answer 12

Malignant neoplasm originating from retina and is the most prevalent intraocular tumour in paeds.

Question 13

Retinoblastoma can be hereditary or non-hereditary. What are they each caused by?

Answer 13

Hereditary:
* Germline mutations in the RB1 gene (a tumour suppressor gene)

Non-hereditary:
* Somatic mutations in the same gene

Question 14

Sx of retinoblastoma?

Answer 14

• Leukocoria (white pupil - pupil appears white)
• Deteriorating vision
• Strabismus
• Failure to thrive
• Eye enlargement

Question 15

What is leukaemia?

Answer 15

Cancer of the stem cells in the bone marrow. This causes unregulated production of certain types of blood cells.

Question 16

What are the different types of leukaemia that affect children from most to least common?

Answer 16

1. Acute lymphoblastic leukaemia (ALL) - MC in children 2-3 yrs
2. Acute myeloid leukaemia (AML) - under 2 yrs
3. Chronic myeloid leukaemia (CML)

Question 17

What does pancytopenia mean?

Answer 17

Combination of low:
* Red blood cells (anaemia)
* White blood cells (leukopenia)
* Platelets (thrombocytopenia)

Question 18

RF for leukaemia?

Answer 18

Main environmental RF: Pregnancy
Several cdtns:
* Down’s syndrome
* Kleinfelter syndrome
* Noonan syndrome
* Fanconi’s anaemia

Question 19

Sx for leukaemia?

Answer 19

• Fever + lethargy
• Failure to thrive
• Weight loss
• Night sweats
• Pallor
• Petechiae and abnormal bruising (thrombocytopenia)
• Unexplained bleeding (thrombocytopenia)
• Generalised lymphadenopathy
• Unexplained or persistent bone or joint pain
• Hepatosplenomegaly

Question 20

Ix for leukaemia?

Answer 20

Nice recommend immediate specialist assess if child has unexplained petechiae or hepatomegaly

• If leukaemia sus - very urgent FBC within 48 hours - can show pancytopenia
• Blood film - shows blast cells
• Bone marrow biopsy
• Lymph node biopsy

Other tests:
* CXR
* CT
* LP
* Genetic analysis

Question 21

Mx of leukaemia?

Answer 21

Primarily: Chemotherapy
Other:
* Radiotherapy
* Bone marrow transplant
* Surgery