Paediatric Oncology Flashcards

1
Q

How common is children’s cancer?

A

Rare

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2
Q

Types of paed malignancy

A

33% leukaemia
25% brain tumours
40% extracranial solid tumours

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3
Q

Is child cancer biologically different from adult cancer?

A

Yes

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4
Q

How do children get cancer?

A

Mostly sporadic

Occasionally genetic basis

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5
Q

What is the 5 year survival for childhood cancer?

A

80%

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6
Q

What is the classification of child cancer based on?

A

Tumour morphology

Primary site

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7
Q

What organisation is used to classify childhood cancer?

A

Internal classification of childhood cancer (ICCC)

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8
Q

What is the most common age to get childhood cancer?

A

0 - 4 y/o

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9
Q

Causes of Childhood cancer

A
Genes
- Downs
- Fanconi 
- BWS
- Li-framumeni familial cancer syndrome
- Neurofibromatosis 
Environmental
- radiation 
- infection - EBV, HPV
Iatrogenic
- chemo 
- radiotherapy
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10
Q

What cancer is downs associated with?

A

Leukaemia

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11
Q

What cancers are Fanconi associated with?

A

Anal / vulval cancers - adult type

Anaemia and developing leukaemia

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12
Q

What cancers are BWS associated with?

A

Hepatoblastomas

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13
Q

What cancers are Li-franumeni familial cancer syndromes associated with?

A

Leukaemia
Sarcomas
Brain tumours

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14
Q

What cancers are neurofibromatosis associated with?

A

Neurofibromas
Sarcomas
Leukaemias

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15
Q

Who needs immediate referral?

A

Unexplained petechiae

Hepatosplenomegaly

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16
Q

Who needs urgent referral within 48 hours?

A
Repeated attendance, same problem, no clear diagnosis
New neuro symptoms
Abdominal mass
Rest pain 
Back pain 
Unexplained lump
Lymphadenopathy
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17
Q

Possible symptoms of childhood cancer

A
Unable to urinate / blood in urine 
Lumps
Swollen glands 
Persistent back pain 
Persistent headaches
Frequent bruising 
Persistent unexplained tiredness
Unexplained seizures or changes in vision or behaviour 
Persistent abdo pain /swelling 
unexplained vomiting
Unexplained sweating or fever
unexplained weight loss or low apetite
Changes in appearance of the eyes or unusual eye reflections in photos
Frequent infections / flu like symptoms
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18
Q

Brain tumour symptoms in children

A

Persistent / recurrent vomiting
Persistent / recurrent headache
Abnormal balance / walking/ coordination
Blurred or double vision
Abnormal eye movements
Behaviour change
Lethargy
Fits / seizures (not with a fever in < 5s)
Abnormal head position
Delayed or arrested puberty in young people

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19
Q

Teenage cancer symptoms

A
Unexplained
Persistent
Pain 
Lump / bump / swelling
Extreme tiredness
Significant weight loss
Changes in a mole
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20
Q

How to find out what cancer it is

A

Scans
Biopsy / pathology
Tumour markers

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21
Q

How to find out where the cancer is

A

Staging e.g. scans, bone marrow

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22
Q

Treatment of childhood cancer

A

Chemotherapy
Surgery
Radiotherapy

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23
Q

Risks of radiotherapy

A
Acute
- lethargy 
- skin irritation 
- swelling
- organ inflammation (bowel / lungs)
Chronic
- fibrosis / scarring
- second cancer
- reduced fertility
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24
Q

Risks of chemo

A
Acute
- hair loss
- nausea and vomiting
- mucositis
- diarrhoea / constipation 
- bone marrow suppression (anaemia, bleeding, infection)
Chronic
- organ impairment (kidneys, hearts, nerves, ears)
- reduced fertility
- second cancer
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25
Q

What are examples of oncological emergencies?

A
Sepsis / febrile neutropenia
Raised ICP
Spinal cord compression 
Mediastinal mass 
Tumour lysis syndrome
26
Q

Risks of sepsis / febrile neutropenia

A

ANC < 0.5x10 9
indwelling catheter
mucosal inflammation
high dose chemo / SCT

27
Q

Causative organisms of febrile neutropenia / sepsis

A

Psueodmonas aeruginosa
Strep pneumoniae
Enterococci
Fungi e.g. Candida

28
Q

Presentations of sepsis / febrile neutropenia in paed oncology

A

Fever / low temp
Rigors
Drowsiness
Shock

29
Q

Presentation of septic shock

A
Tachycardia
Tachypnoea
Hypotension 
Prolonged cap refill time 
reduced UO
Metabolic acidosis
30
Q

Investigations of febrile neutropenia / sepsis

A
IV access
Blood culture / FBC / U and Es, coagulation / LFTs, CRP, lactate
CXR
urine microscopy / culture
Throat swab 
Sputum culture / BAL 
LP 
viral PCRs
CT / USS
31
Q

Treatment of sepsis / febrile neutropenia

A
ABC 
- oxygen 
- fluids
Broad spectrum Ax
Inotropes
PICU
32
Q

When is raised ICP uncommon?

A

In children < 1 y/o due to fontanelles

33
Q

Presentation of raised ICP

A
Early morning headache / vomiting 
Tense fontanelle
Increasing head circumference disproportionate to body 
Late
- constant headache
- papilloedema
- diplopia
- loss of up gaze
- neck stiffness
- status elipticus
- reduced GCS
- cushings triad (low HR, high BP)
34
Q

Investigations for raised ICP

A

CT

MRI (more accurate)

35
Q

Treatment of raised ICP

A
If due to tumour = dexamethasone 
For urgent CSF diversion = neurosurgery 
- ventriculostomy 
- EVD (temporary)
- VD shunt
36
Q

What is spinal cord compression a potential malignancy of?

A

nearly all paediatric malignancies

37
Q

Pathological processes of spinal cord compression in children

A
Invasion of paravertebral disease via intervertebral foramina (40% extradural)
Vertebral body compression (30%)
CSF seeding (20% intradural, extraspinal)
Direct invasion (10% intraspinal)
38
Q

Presentations of spinal cord compression

A

Symptoms vary with level

  • weakness (90%)
  • pain (55-95%)
  • Sensory (10-55%)
  • sphincter disturbance (10-35%)
39
Q

Treatment of Spinal cord compression

A
Urgent MRI
Dexamethasone urgently to reduce peri tumour oedema
Chemotherapy  
Surgery 
Radiotherapy
40
Q

Prognosis of spinal cord compression

A

Mild impairment > 90% recovery

Paraplegia 65% recovery

41
Q

What does the prognosis of spinal cord compression depend on?

A

The severity of impairment

42
Q

Common causes of SVC syndrome / SMS

A

lymphoma
neuroblastoma
germ cell tumour
thrombosis

43
Q

Presentation of SVCS

A
facial, neck and upper thoracic plethora 
Oedema
Cyanosis 
Distended veins
ill 
anxious 
reduced GCS
44
Q

Presentation of SMS

A
Dyspnoea
Tachypnoea
Cough 
Wheeze
Stridor
Orthopnoea
45
Q

Investigations of SVC syndrome / SMS

A
CXR / CT chest
ECHO
Urgent biopsy 
FBC, BM, Pleural aspirate
GCT markers
46
Q

Treatment of SVC syndrome / SMS

A

Keep upright in position they are comfortable in and calm
Chemotherapy
Steroids may be needed in the absence of a histological diagnosis
Radiotherapy
Surgery - rarely if infinitive

47
Q

Pathology of tumour lysis syndrome

A

Metabolic derangement
Rapid death of tumour cells
Release of intracellular contents
At or shortly after presentation

48
Q

What is tumour lysis syndrome secondary to?

A

Treatment

Rarely spontaenous

49
Q

Presentation of tumour lysis syndrome

A
Increased K
Increased urate, relatively insoluble
Increased P
Decreased Ca
Acute renal failure
- urate load 
- CoPO4 deposition in renal tubules
50
Q

Treatment of tumour lysis syndrome

A
Avoidance
ECG monitoring 
Hyperhydrate 
QDS electrolytes 
Diuresis 
Decrease uric acid
- allopurinol 
- urate oxidase 
Treat hyperkalaemia
 - Ca resonium 
- salbutamol 
- insulin 
Renal replacement therapy
51
Q

What do you NEVER give in tumour lysis syndrome?

A

Potassium

52
Q

What do you check the red reflex in babies for?

A

Retinoblastoma

Congenital cataract

53
Q

Poor prognostic factors for ALL

A
Male sex
Age < 2 or > 10 
WBC > 20 x 10^9 at diagnosis
T or B cell surface markers
Non Caucasian
54
Q

What is the most common ocular malignancy found in childhood?

A

Retinoblastoma

55
Q

Inheritance of retinoblastoma and what % are inherited?

A

Autosomal dominant

10% inherited

56
Q

Pathology of retinoblastoma

A

Loss of function of the retinoblastoma tumour suppressor gene on chromosome 13

57
Q

Possible presentations of retinoblastoma

A

Absence of red reflex, replaced by a white pupil (leukocoria)
Strabismus
Visual problems

58
Q

What is the most common presentation of retinoblastoma?

A

Absence of red reflex and replacement with a white pupil (leukocoria)

59
Q

Treatment of retinoblastoma

A

Enucleation
External beam radiation therapy
Chemo
Photocoagulation

60
Q

Prognosis of retinoblastoma

A

Excellent - > 90% survive into adulthood