Paediatric Oncology Flashcards

1
Q

What is the incidence of childhood cancer?

A

1:500 before 14

Slightly more common in boys

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2
Q

What are the major classes of paediatric cancers?

A

Leukaemias
Brain tumours
Extra-cranial solid tumours= rest grouped together

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3
Q

What portion of paediatric cancers have a genetic component?

A

~10%

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4
Q

What is the overall 5-year survival rate for paediatric cancer?

A

80%

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5
Q

In what age groups are paediatric cancers most common?

A

Very young children

Adolescents

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6
Q

What are the possible causes of childhood cancer?

A
Genes:
-Down
-Fancomi
-BWS
-Li-Fraumeni familial cancer syndrome
-Neurofibromatosis
Environment:
-Radiation 
-Infection
Iatrogenic:
-Chemotherapy
-Radiotherapy
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7
Q

What presentations require immediate, urgent and general referral to investigate for childhood cancer?

A
IMMEDIATE referral:
-Unexplained petechiae
-Hepatosplenomegaly
Urgent referral:
-Repeat attendance with same problem and no resolution
-New neurological symptoms
-Abdominal mass
General Referral:
-Pain at rest
-Back pain
-Unexplained lump
-Lymphadenopathy
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8
Q

How do oncologists investigate suspected childhood cancer?

A

Scans- MRI gold standard (GA needed for MRI in children as they won’t stay still- difficult to arrange urgent scan + urgent anaesthesia so CT sometime used instead)
Biopsy/pathology
Tumour markers
Staging with scans (CT chest, bone scans PET scan) and bone marrow aspirate

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9
Q

How is paediatric malignancy managed?

A

Paediatric malignancy is managed with an MDT approach regarding chemotherapy, radiotherapy and surgery

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10
Q

What are the acute side effects of chemotherapy in children?

A
Hair loss
N&V
Mucositis
Diarrhoea or constipation
Bone marrow suppression- anaemia, bleeding, infection
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11
Q

What are the chronic side effects of chemotherapy in children?

A

Organ impairment- kidneys, heart, nerve, hearing
Reduced fertility
Second cancer

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12
Q

What are the acute side effects of radiotherapy?

A

Lethargy
Skin irritation
Swelling
Organ inflammation- bowel, lungs

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13
Q

What are the chronic side effects of radiotherapy?

A

Fibrosis/scarring
Second cancer
Reduced fertility

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14
Q

What are the different paediatric oncological emergencies?

A
Sepsis/febrile neutropenia
Raised ICP
Spinal cord compression
Mediastinal mass
Tumour lysis syndrome
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15
Q

What are the risk factors for febrile neutropenia in paediatric oncology?

A
ANC < 0.5 x 109 
Indwelling catheter
Mucosal inflammation
High dose chemotherapy
Stem cell transplant
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16
Q

What are the possible causative organisms of febrile neutropenia in paediatric oncology?

A
Pseudomonas aeruginosa
Enterobacteriaciae eg E coli, Klebsiella
Streptococcus pneumoniae
Enterococci
Staphylococcus 
Fungi eg. Candida, Aspergillus
17
Q

How does febrile neutropenia present?

A
Fever >38 degrees
Rigors (marker of serious infection)
Drowsiness
Shock:
-Tachycardia
-Tachypnoea
-Hypotension
-Prolonged capillary refill time
-Metabolic acidosis
-Decreased urine output
18
Q

How is febrile neutropenia managed?

A
Gain IV access
Blood culture, FBC, coagulations, Us+Es, LFTs, CRP and lactate
Chest x-ray
Other investigations as appropriate
Oxygen
Fluids
Broad spectrum Abx
Inotropes
19
Q

What are the early features of raised ICP?

A

Early morning headache or vomiting

Tense fontanelle

20
Q

What are the late signs of raised ICP?

A
Constant headache
Papilloedema
Diplopia- CNVI palsy
Loss of upgaze
Neck stiffness
Status epilepticus
Reduced GCS
Cushings triad- low HR, high BP, reduced RR
21
Q

How is raised ICP investigated and managed?

A

Imaging mandatory- MRI gold standard but can use CT
Management:
•Dexamethasone- reduces oedema and increases CSF flow- buys time
•Neurosurgery- urgent CSF diversion via ventriculostomy, EVD or VP shunt

22
Q

What is the incidence of spinal cord compression?

A

Affects ~5% of children with cancer
Can be associated with most kinds of cancer
Most common in Ewing’s sarcoma

23
Q

What pathological processes are associated with spinal cord compression?

A

Invasion from paravertebral disease via intervertebral foramina
Vertebral body compression
CSF seeding
Direct invasion

24
Q

What are the symptoms of spinal cord compression?

A

Weakness
Pain
Sensory dysfunction
Sphincter disturbance

25
Q

How is spinal cord compression managed?

A

Urgent MRI
Start dexamethasone urgently to reduce peri-tumoral oedema
Definitive treatment with chemotherapy appropriate if a rapid response is expected (surgery or radiotherapy also options)

26
Q

What are the causes of superior vena cava syndrome?

A

Lymphoma
Neuroblastoma
Germ cell tumours

27
Q

What is the presentation of superior vena cava syndrome?

A
Facial, neck and upper thoracic plethora
Oedema
Cyanosis
Distended veins
Ill and anxious
Reduced GCS
28
Q

How is superior vena cava syndrome investigated?

A

Chest x-ray
CT
Echo

29
Q

How is superior vena cava syndrome managed?

A

Keep patient upright and calm
Urgent biopsy
FBC, pleural aspirate, GCT markers
Definitive treatment required urgently- chemotherapy, steroids and radiotherapy can be helpful

30
Q

What is tumour lysis syndrome?

A

Tumour lysis syndrome occurs as a result of metabolic derangement resulting in the rapid death of tumour cells, with the release of intracellular contents. This usually occurs secondary to treatment and is very rarely spontaneous

31
Q

In what cancers is tumour lysis syndrome most common?

A

Burkitt’s lymphoma

High count leukaemias

32
Q

What are the clinical features of tumour lysis syndrome?

A
Increased potassium
Elevated urate
Increased phosphate
Decreased calcium
Acute renal failure
33
Q

How is tumour lysis syndrome treated?

A

ECG monitoring
Hyperhydration with fluid
Electrolytes
Diuresis
Avoidance of potassium (even if slightly hypokalemic do not give potassium)
Reduce uric acid levels (allopurinol, urate oxidase-uricozyme)
Treat hyperkalaemia (Ca resonium, salbutamol, insulin)
Renal replacement therapy