Paediatric, teenage and young adult

Question 1

Causative factors

Answer 1

No definite and known causes

Question 2

Genetic causative factors

Answer 2

Very rare - possibly linked to retinoblastoma

Some children born with genetic changes that may increase risk of developing a malignancy

Down’s syndrome (10-20% increased risk of acute leukaemia’s but lessened risk of developing solid tumours)

Neurofibromatosis

Question 3

Environmental causative factors

Answer 3

Radiation
Pelvic imaging during pregnancy linked to ~30% increased risk of leukaemia/ solid tumour in childhood

No evidence that environmental radiation increases risk

EMF (no evidence)

Question 4

In the 0-14 age group what percentage of cancers are leukaemia?

Answer 4

1/3 of diagnoses

Question 5

In the 0-14 age group what percentage of cancers are leukaemia, brain and lymphoma?

Answer 5

2/3 of diagnoses

Question 6

Childhood cancers examples

Answer 6

Retinoblastoma, rhabdomyosarcoma, neuroblastoma, wilm’s tumour, medulloblastoma, DIPG

Question 7

Retinoblastoma

Answer 7

Optic nerve invaded
Children under 5
Genetic - RB1 mutation
Leukocoria - white glow/refelction
Ophthalomoscopy
Intraocular/extraocular

Chemo/RT/surgery

Question 8

Rhabdomyosarcoma

Answer 8

Muscle tissue cancer
Children under 10 - boys
H&N, bladder, testes, uterus
Embryonal/Alveolar/Pleomorphic

Question 9

Rhabdomyosarcoma - Embryonal location and treatment

Answer 9

Head and neck, bladder or genital area

Surgery and chemo

Question 10

Rhabdomyosarcoma - Alveolar location and treatment

Answer 10

Arms and legs of older children, can also occur in trunk

Surgery, chemo, RT

Question 11

Rhabdomyosarcoma - Pleomorphic location and treatment

Answer 11

Adults
Surgery and RT (does not respond well to chemo)

Question 12

Neuroblastoma

Answer 12

Develops in neuroblasts - early nerve cells
Commonly abdomen - adrenal glands
Age 5
Blood tests, US abdomen, chest, MRI, PET-CT

Surgery, chemo, RT, immunotherapy (GD2 MAB used- dinutuximab)

Question 13

Wilm’s tumour

Answer 13

Nephroblastoma, kidney tumour
Under 5s - girls
Usually 1 kidney but 10% cases 2
Blood test, US, Chest imaging, MRI, biopsy

Chemo, surgery (nephrectomy), RT

Question 14

Medulloblastoma

Answer 14

15-20% of childhood brain tumours
More common in ages 3- 8 years
(2nd most common brain tumour, 1st most common malignant brain tumour - astrocytoma most common)
Fast growing

4 subgroups:
WNT - regulate cell growth, motility and differentiation during embryonic development
SHH - TP53 status
Group 3 - aggressive, worst prognosis
Group 4 - most common

Headaches, morning vomiting, vision issues, bowel/bladder control

Surgery (reduce pressure), RT whole brain/spinal cord/whole CNS, chemo

70-80% survival at 5 years + (subtype dependant)

Question 15

What does DIPG stand for?

Answer 15

Diffuse Intrinsic Pontine Glioma

Question 16

What is DIPG and where does it originate?

Answer 16

A very high grade Glioma
Develops in the Pons
The Pons is the area of the brain stem responsible for critical functions; breathing, sleeping, blood pressure

Question 17

DIPG symtoms

Answer 17

Abnormal eye alignment
Weakness of facial muscles/ facial asymmetry
Arm/ leg weakness
Unstable balance

Question 18

DIPG management

Answer 18

No cure! RT
Surgery not always an option due to position
Chemotherapy drugs are not shown to be effective, no increase in overall survival than RT alone.

TRIALS - ACVR1 gene

Question 19

DIPG prognosis

Answer 19

90% mortality within 18 months of diagnosis
2 year survival is extremely rare

Question 20

Most common 0-4

Answer 20

Acute Lymphoblastic Leukaemia
Retinoblastoma
Neuroblastoma
Wilm’s tumor (seldom present at birth)
Hepatoblastoma
Embryonal rhabdomyosarcoma

Question 21

Most common 5-9

Answer 21

Acute Lymphoblastic Leukaemia
Retinoblastoma
Medulloblastoma
Ewing’s sarcoma
Juvenile pilocytic astrocytoma (infratentorial)

Question 22

Most common 10-14

Answer 22

Hodgkin’s disease
Osteosarcoma
Alveolar rhabdomyosarcoma
Papillary carcinoma of the thyroid