paediatric oncology Flashcards

1
Q

what is cancer

A

abnormal cells dividing in an uncontrolled way

gene changes

stimulates own blood supply

local invasion

metastatic spread via blood or lymphatic systems

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2
Q

how common is childhood cancer

A

rare - 1821 cases <15 in UK p/a (130 scotland)

1/500 pre age 14

<1% all cancers

M>F

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3
Q

classification of childhood cancer

A

based on tumour morphology (and primary site) - cell of origin

standard classification is essential for comparing incidence and survival across regions and over time periods

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4
Q

what cancers do children get

A

31% leukaemia - mainly acute lymphoblastic leukaemia

26% CNS tumours - increases in teenage yrs

10% lymphoma

7% soft tissue tumours

6% neuroblastoma

5% renal tumours

4% malignant bone tumours

4% other

3% germ cell tumours

3% retinoblastomas

1% hepatic tumours

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5
Q

when do children get cancer

A

more likely at a younger age (0-4)

decreases between 5-14

2nd peak at 15-24 - increased risk of lymphomas and germ cell tumours

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6
Q

why do children get cancer

A

genetic - down (leukaemia), fanconi, BWS (neuro and nephroblastoma, hepatoblastoma), Li-Fraumeni familial cancer syndrome (strong FHx of cancer), neurofibromatosis (soft tissue tumours and nerve sheath tumours, brain tumours)

environment - radiation, infection (EBV - burkitt’s lymphoma)

iatrogenic - chemo and radiotherapy

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7
Q

diagnostic journey of cancer

A
  • biological onset of disease
  • symptom onset
  • seek medical attention
  • doctor recognises cancer as a possibility
  • investigation, diagnosis, treatment
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8
Q

why are there delays in cancer diagnosis

A

patient doesn’t seek medical attention

doctor doesn’t recognise cancer as a possibility

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9
Q

what children should you be worried about - when to refer

A
  • immediate referral
    • unexplained petechiae,
    • hepatosplenomegaly
  • urgent referral
    • repeat attendance, same problem, no clear diagnosis
    • new neuro symptoms, abdo mass
  • refer (to doctor for urgent investigations)
    • rest pain, back pain and unexplained lump
    • lymphadenopathy (>1cm, growing continuously, firm, rubbery, not mobile, not associated w/ infective symptoms )
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10
Q

symptoms to see a doctor about

how often are they cancer

A

3/1000 - not able to wee, blood in wee, unexplained lump/firmness anywhere on the body

1/1000 - lymphadenopathy, frequent bruising, persistent back pain, persistent unexplained tiredness, persistent headaches, unexplained seizures or changes in vision or behaviour

persistent abdo swelling, unexplained vomiting, unexplained sweating/fever, unexplained weight loss/low appetite, changes in appearance of eyes or unusual reflections in photos, frequent infections or flu like symptoms

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11
Q

symptoms of brain tumours in pre-school children

A

persistent/recurrent vomiting

abnormal balance/walking/coordination

abnormal eye movements

behaviour change esp lethargy

fits/seizures (w/o fever)

abnormal head position - wry neck, head tilt, stiff neck

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12
Q

symptoms of brain tumours in 5-11y/o

A

persistent/recurrent vomiting

persistent/recurrent headache

abnormal balance/walking/coordination

abnormal eye movements

blurred/double vision

behaviour change

fits/seizures

abnormal head position - wry neck, head tilt, stiff neck

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13
Q

symptoms of brain tumours in 12-18y/o

A

persistent/recurrent vomiting

persistent/recurrent headache

abnormal balance/walking/coordination

abnormal eye movements

blurred/double vision

behaviour change

fits/seizures

delayed/arrested puberty

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14
Q

what are some oncological emergencies

A
  • can be at diagnosis or a consequence of treatment:

sepsis/febrile neutropenia

raised ICP

spinal cord compression

mediastinal mass

tumour lysis syndrome

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15
Q

sepsis/febrile neutropenia - why is it important

A
  • infection is a major cause of morbidity/mortality
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16
Q

sepsis/febrile neutropenia - risk factors

A
  • ANC <0.5 x109
  • indwelling catheter
  • mucosal inflammation
  • high dose chemo/SCT
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17
Q

sepsis/febrile neutropenia - causative organisms

A
  • pseudomonas aeruginosa
  • enterobacteriae e.g. e. coli, klebsiella
  • streptococcus pnuemoniae
  • enterococci
  • staphylococcus
  • fungi e.g. candida, aspergillus
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18
Q

sepsis/febrile neutropenia - presentation

A
  • fever/low temp
  • rigors
  • drowsiness
  • shock - tachycardia, tachypnoea, hypotension, prolonged CRT, reduced UO, metabolic acidosis
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19
Q

sepsis/febrile neutropenia - management

A
  • IV access
  • bloods: culture, FBC, coag, U+E, LFTs, CRP, lactate
  • CXR - evidence of pneumonia/fungal infection
  • other - urine microscopy/culture, throat swab, sputum culture/BAL, LP, viral PCR, CT/USS (abscesses, deep seated infection)
  • ABC - oxygen, fluids
  • broad spectrum abx
  • inotropes
  • PICU
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20
Q

early presentation of raised ICP

A

early morning headache/vomiting

tense fontanelle

increasing head circumferece

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21
Q

late presentation of raised ICP

A

constant headache

papilloedema

diplopia (VI palsy)

loss of upgaze

neck stiffness

status epilepticus

reduced GCS

cushing’s triad (low HR, high BP, falling RR)

22
Q

investigation of raised ICP

A

imaging is mandatory (if safe)

  • CT good for screening
  • MRI best for more accurate diagnosis
23
Q

management of raised ICP

A

dexamethasone if due to tumour

  • reduce oedema and increased CSF flow
  • 250mcg/kg iV stat and then 125mcg/kg BD

NEUROSURGERY - urgent CSF diversion

  • ventriculostomy - hole in membrane at base of 3rd ventricle w/ endoscope
  • EVD (temporary)
  • VP shunt
24
Q

how common is spinal cord compression

A
  • potential complication w/ nearly all paeds malignancies
    • 5% of all children w/ cancer
    • 10-20% ewing’s or medulloblastoma
    • 5-10% neuroblastoma and germ cell tumour
25
Q

pathological process of spinal cord compression

A
  • invasion from paravertebral disease via intervertebral foramina (40% extradural)
  • vertebral body compression (30%)
  • CSF seeding (20% intradural, extraspinal)
  • direct invasion (10% intraspinal)
26
Q

presentation of spinal cord compression

A

symptoms vary w/ level

  • weakness 90%
  • pain 55-95%
  • sensory 10-55%
  • sphincter disturbance 10-35%
27
Q

management of spinal cord compression

A
  • urgent MRI
  • start dexamethasone urgently to reduce peri-tumour oedema
  • definitive treatment w/ chemo is appropriate when rapid response expected
    • surgery or RT are other options
28
Q

outcome from spinal cord compression

A

outcome depends on severity of impingement rather than duration between symptoms and diagnosis

  • mild impairment >90% recovery
  • paraplegic 65% recovery
29
Q

superior vena cava syndrome (aka superior mediastinum syndrome) - how common and what are the causes

A

<1% of new paeds malignancies

lymphoma, other (neuroblastoma, germ cell tumour, thrombosis)

30
Q

presentation of SVC syndrome

A

SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS

SMS: SOB, tachypnoea, cough, wheeze, stridor, orthopnoea

31
Q

investigation for SVC/SMS syndrome

A

CXR/CT if able to tolerate

echo

32
Q

management of SVC/SMS syndrome

A
  • keep upright and calm
  • urgent biopsy (ideally)
  • try to obtain diagnostic info w/o GA
    • FBC, BM, pleural aspirate, GCT markers
  • definitive treatment is required urgently
33
Q

definitive treatment of SVC/SMS syndrome

A
  • chemo usually rapidly effective
  • presumptive treatment may be needed in the absence of a definitive histological diagnosis - steroids
  • RT effective
    • may cause initial increased resp distress
  • rarely surgery if insensitive
  • CVAD associated thrombosis should be treated by thrombolytic therapy
34
Q

what is tumour lysis syndrome

A

metabolic derangement

rapid death of tumour cells

release of intracellular components

  • at/shortly after presentation, 2y to treatment (rarely spontaneous)
35
Q

clinical features of tumour lysis syndrome

A
  • increased K
  • increased urate - relatively insoluble
  • increased phosphate
  • reduced Ca

→ acute renal failure (urate load, calcium phosphate deposition in renal tubules)

36
Q

management of tumour lysis syndrome

A
  • avoid developing it
  • ECG monitoring
  • hyperhydrate - 2.5ml/m2
  • QDS electrolytes
  • diuresis
  • NEVER GIVE POTASSIUM
  • reduce uric acid - urate oxidase-uricoenzyme (rasburicase), allopurinol
  • treat hyperkalaemia - Ca resonium, salbutamol, insulin
  • renal replacement therapy
37
Q

how to find out what the tumour is and what is causing it

A
  • scans - MRI preferable but requires GA, CT used occasionally
  • biopsy/pathology - info about cell of origin
  • cytogenetics
  • tumour markers
38
Q

staging tumours

A
  • where is it - staging e.g. scans (CXR, bone scans), bone marrow
39
Q

treatment of cancer - principles

A
  • multimodal therapy based on specific disease and extent - plus pt factors
  • MDT approach
  • national/international collaboration
  • clinical research
40
Q

treatment options for cancer

A
  • surgical resection
  • chemotherapy
  • radiotherapy
  • immunotherapy
  • bone marrow and stem cell transplant
41
Q

acute risks of chemotherapy

A
  • hair loss
  • N+V
  • mucositis
  • diarrhoea/constipation
  • bone marrow suppression - anaemia, bleeding, infection
42
Q

chronic risks of chemotherapy

A

organ impairment - kidneys, heart, nerves, ears

reduced fertility

2nd cancer

43
Q

acute risks of radiotherapy

A
  • lethargy
  • skin irritation
  • swelling
  • organ inflammation - bowel, lungs
44
Q

chronic risks of radiotherapy

A
  • fibrosis/scarring
  • 2nd cancer
  • reduced fertility
45
Q

late effects of childhood and adolescent cancer

A
  • growth and development
  • organ function
  • fertility and reproduction
  • cancer
  • psychosocial
46
Q

late effects of childhood and adolescent cancer - growth and development

A
  • skeletal maturation
  • linear growth
  • emotional and social maturation
  • intellectual function
  • sexual development
47
Q

late effects of childhood and adolescent cancer - organ function

A
  • cardiac
  • endo
  • GI and hepatic
  • genitourinary
  • MSK
  • neuro
  • pulmonary
48
Q

late effects of childhood and adolescent cancer - fertility and reproduction

A
  • fertility
  • health of offspring
  • sexual functioning
49
Q

late effects of childhood and adolescent cancer - cancer

A

recurrent 1y cancer

subsequent neoplasms

50
Q

late effects of childhood and adolescent cancer - psychosocial

A
  • mental health
  • education
  • employment
  • health insurance
  • chronic symptoms
  • physical/body image