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
What are examples of oncological emergencies?
``` Sepsis / febrile neutropenia Raised ICP Spinal cord compression Mediastinal mass Tumour lysis syndrome ```
26
Risks of sepsis / febrile neutropenia
ANC < 0.5x10 9 indwelling catheter mucosal inflammation high dose chemo / SCT
27
Causative organisms of febrile neutropenia / sepsis
Psueodmonas aeruginosa Strep pneumoniae Enterococci Fungi e.g. Candida
28
Presentations of sepsis / febrile neutropenia in paed oncology
Fever / low temp Rigors Drowsiness Shock
29
Presentation of septic shock
``` Tachycardia Tachypnoea Hypotension Prolonged cap refill time reduced UO Metabolic acidosis ```
30
Investigations of febrile neutropenia / sepsis
``` 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
Treatment of sepsis / febrile neutropenia
``` ABC - oxygen - fluids Broad spectrum Ax Inotropes PICU ```
32
When is raised ICP uncommon?
In children < 1 y/o due to fontanelles
33
Presentation of raised ICP
``` 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
Investigations for raised ICP
CT | MRI (more accurate)
35
Treatment of raised ICP
``` If due to tumour = dexamethasone For urgent CSF diversion = neurosurgery - ventriculostomy - EVD (temporary) - VD shunt ```
36
What is spinal cord compression a potential malignancy of?
nearly all paediatric malignancies
37
Pathological processes of spinal cord compression in children
``` Invasion of paravertebral disease via intervertebral foramina (40% extradural) Vertebral body compression (30%) CSF seeding (20% intradural, extraspinal) Direct invasion (10% intraspinal) ```
38
Presentations of spinal cord compression
Symptoms vary with level - weakness (90%) - pain (55-95%) - Sensory (10-55%) - sphincter disturbance (10-35%)
39
Treatment of Spinal cord compression
``` Urgent MRI Dexamethasone urgently to reduce peri tumour oedema Chemotherapy Surgery Radiotherapy ```
40
Prognosis of spinal cord compression
Mild impairment > 90% recovery | Paraplegia 65% recovery
41
What does the prognosis of spinal cord compression depend on?
The severity of impairment
42
Common causes of SVC syndrome / SMS
lymphoma neuroblastoma germ cell tumour thrombosis
43
Presentation of SVCS
``` facial, neck and upper thoracic plethora Oedema Cyanosis Distended veins ill anxious reduced GCS ```
44
Presentation of SMS
``` Dyspnoea Tachypnoea Cough Wheeze Stridor Orthopnoea ```
45
Investigations of SVC syndrome / SMS
``` CXR / CT chest ECHO Urgent biopsy FBC, BM, Pleural aspirate GCT markers ```
46
Treatment of SVC syndrome / SMS
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
Pathology of tumour lysis syndrome
Metabolic derangement Rapid death of tumour cells Release of intracellular contents At or shortly after presentation
48
What is tumour lysis syndrome secondary to?
Treatment | Rarely spontaenous
49
Presentation of tumour lysis syndrome
``` Increased K Increased urate, relatively insoluble Increased P Decreased Ca Acute renal failure - urate load - CoPO4 deposition in renal tubules ```
50
Treatment of tumour lysis syndrome
``` Avoidance ECG monitoring Hyperhydrate QDS electrolytes Diuresis Decrease uric acid - allopurinol - urate oxidase Treat hyperkalaemia - Ca resonium - salbutamol - insulin Renal replacement therapy ```
51
What do you NEVER give in tumour lysis syndrome?
Potassium
52
What do you check the red reflex in babies for?
Retinoblastoma | Congenital cataract
53
Poor prognostic factors for ALL
``` Male sex Age < 2 or > 10 WBC > 20 x 10^9 at diagnosis T or B cell surface markers Non Caucasian ```
54
What is the most common ocular malignancy found in childhood?
Retinoblastoma
55
Inheritance of retinoblastoma and what % are inherited?
Autosomal dominant | 10% inherited
56
Pathology of retinoblastoma
Loss of function of the retinoblastoma tumour suppressor gene on chromosome 13
57
Possible presentations of retinoblastoma
Absence of red reflex, replaced by a white pupil (leukocoria) Strabismus Visual problems
58
What is the most common presentation of retinoblastoma?
Absence of red reflex and replacement with a white pupil (leukocoria)
59
Treatment of retinoblastoma
Enucleation External beam radiation therapy Chemo Photocoagulation
60
Prognosis of retinoblastoma
Excellent - > 90% survive into adulthood