paediatric oncology Flashcards

(50 cards)

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
pathological process of spinal cord compression
* invasion from paravertebral disease via intervertebral foramina (40% extradural) * vertebral body compression (30%) * CSF seeding (20% intradural, extraspinal) * direct invasion (10% intraspinal)
26
presentation of spinal cord compression
symptoms vary w/ level * weakness 90% * pain 55-95% * sensory 10-55% * sphincter disturbance 10-35%
27
management of spinal cord compression
* 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
outcome from spinal cord compression
outcome depends on severity of impingement rather than duration between symptoms and diagnosis * mild impairment \>90% recovery * paraplegic 65% recovery
29
superior vena cava syndrome (aka superior mediastinum syndrome) - how common and what are the causes
\<1% of new paeds malignancies lymphoma, other (neuroblastoma, germ cell tumour, thrombosis)
30
presentation of SVC syndrome
SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS SMS: SOB, tachypnoea, cough, wheeze, stridor, orthopnoea
31
investigation for SVC/SMS syndrome
CXR/CT if able to tolerate echo
32
management of SVC/SMS syndrome
* 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
definitive treatment of SVC/SMS syndrome
* **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
what is tumour lysis syndrome
metabolic derangement rapid death of tumour cells release of intracellular components * at/shortly after presentation, 2y to treatment (rarely spontaneous)
35
clinical features of tumour lysis syndrome
* increased K * increased urate - relatively insoluble * increased phosphate * reduced Ca → acute renal failure (urate load, calcium phosphate deposition in renal tubules)
36
management of tumour lysis syndrome
* 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
how to find out what the tumour is and what is causing it
* scans - MRI preferable but requires GA, CT used occasionally * biopsy/pathology - info about cell of origin * cytogenetics * tumour markers
38
staging tumours
* where is it - staging e.g. scans (CXR, bone scans), bone marrow
39
treatment of cancer - principles
* multimodal therapy based on specific disease and extent - plus pt factors * MDT approach * national/international collaboration * clinical research
40
treatment options for cancer
* surgical resection * chemotherapy * radiotherapy * immunotherapy * bone marrow and stem cell transplant
41
acute risks of chemotherapy
* hair loss * N+V * mucositis * diarrhoea/constipation * bone marrow suppression - anaemia, bleeding, infection
42
chronic risks of chemotherapy
organ impairment - kidneys, heart, nerves, ears reduced fertility 2nd cancer
43
acute risks of radiotherapy
* lethargy * skin irritation * swelling * organ inflammation - bowel, lungs
44
chronic risks of radiotherapy
* fibrosis/scarring * 2nd cancer * reduced fertility
45
late effects of childhood and adolescent cancer
* growth and development * organ function * fertility and reproduction * cancer * psychosocial
46
late effects of childhood and adolescent cancer - growth and development
* skeletal maturation * linear growth * emotional and social maturation * intellectual function * sexual development
47
late effects of childhood and adolescent cancer - organ function
* cardiac * endo * GI and hepatic * genitourinary * MSK * neuro * pulmonary
48
late effects of childhood and adolescent cancer - fertility and reproduction
* fertility * health of offspring * sexual functioning
49
late effects of childhood and adolescent cancer - cancer
recurrent 1y cancer subsequent neoplasms
50
late effects of childhood and adolescent cancer - psychosocial
* mental health * education * employment * health insurance * chronic symptoms * physical/body image