paediatric oncology Flashcards
what is cancer
abnormal cells dividing in an uncontrolled way
gene changes
stimulates own blood supply
local invasion
metastatic spread via blood or lymphatic systems
how common is childhood cancer
rare - 1821 cases <15 in UK p/a (130 scotland)
1/500 pre age 14
<1% all cancers
M>F
classification of childhood cancer
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
what cancers do children get
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
when do children get cancer
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
why do children get cancer
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
diagnostic journey of cancer
- biological onset of disease
- symptom onset
- seek medical attention
- doctor recognises cancer as a possibility
- investigation, diagnosis, treatment
why are there delays in cancer diagnosis
patient doesn’t seek medical attention
doctor doesn’t recognise cancer as a possibility
what children should you be worried about - when to refer
-
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 )
symptoms to see a doctor about
how often are they cancer
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
symptoms of brain tumours in pre-school children
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
symptoms of brain tumours in 5-11y/o
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
symptoms of brain tumours in 12-18y/o
persistent/recurrent vomiting
persistent/recurrent headache
abnormal balance/walking/coordination
abnormal eye movements
blurred/double vision
behaviour change
fits/seizures
delayed/arrested puberty
what are some oncological emergencies
- can be at diagnosis or a consequence of treatment:
sepsis/febrile neutropenia
raised ICP
spinal cord compression
mediastinal mass
tumour lysis syndrome
sepsis/febrile neutropenia - why is it important
- infection is a major cause of morbidity/mortality
sepsis/febrile neutropenia - risk factors
- ANC <0.5 x109
- indwelling catheter
- mucosal inflammation
- high dose chemo/SCT
sepsis/febrile neutropenia - causative organisms
- pseudomonas aeruginosa
- enterobacteriae e.g. e. coli, klebsiella
- streptococcus pnuemoniae
- enterococci
- staphylococcus
- fungi e.g. candida, aspergillus
sepsis/febrile neutropenia - presentation
- fever/low temp
- rigors
- drowsiness
- shock - tachycardia, tachypnoea, hypotension, prolonged CRT, reduced UO, metabolic acidosis
sepsis/febrile neutropenia - management
- 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
early presentation of raised ICP
early morning headache/vomiting
tense fontanelle
increasing head circumferece
late presentation of raised ICP
constant headache
papilloedema
diplopia (VI palsy)
loss of upgaze
neck stiffness
status epilepticus
reduced GCS
cushing’s triad (low HR, high BP, falling RR)
investigation of raised ICP
imaging is mandatory (if safe)
- CT good for screening
- MRI best for more accurate diagnosis
management of raised ICP
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
how common is spinal cord compression
- 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
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)
presentation of spinal cord compression
symptoms vary w/ level
- weakness 90%
- pain 55-95%
- sensory 10-55%
- sphincter disturbance 10-35%
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
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
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)
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
investigation for SVC/SMS syndrome
CXR/CT if able to tolerate
echo
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
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
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)
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)
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
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
staging tumours
- where is it - staging e.g. scans (CXR, bone scans), bone marrow
treatment of cancer - principles
- multimodal therapy based on specific disease and extent - plus pt factors
- MDT approach
- national/international collaboration
- clinical research
treatment options for cancer
- surgical resection
- chemotherapy
- radiotherapy
- immunotherapy
- bone marrow and stem cell transplant
acute risks of chemotherapy
- hair loss
- N+V
- mucositis
- diarrhoea/constipation
- bone marrow suppression - anaemia, bleeding, infection
chronic risks of chemotherapy
organ impairment - kidneys, heart, nerves, ears
reduced fertility
2nd cancer
acute risks of radiotherapy
- lethargy
- skin irritation
- swelling
- organ inflammation - bowel, lungs
chronic risks of radiotherapy
- fibrosis/scarring
- 2nd cancer
- reduced fertility
late effects of childhood and adolescent cancer
- growth and development
- organ function
- fertility and reproduction
- cancer
- psychosocial
late effects of childhood and adolescent cancer - growth and development
- skeletal maturation
- linear growth
- emotional and social maturation
- intellectual function
- sexual development
late effects of childhood and adolescent cancer - organ function
- cardiac
- endo
- GI and hepatic
- genitourinary
- MSK
- neuro
- pulmonary
late effects of childhood and adolescent cancer - fertility and reproduction
- fertility
- health of offspring
- sexual functioning
late effects of childhood and adolescent cancer - cancer
recurrent 1y cancer
subsequent neoplasms
late effects of childhood and adolescent cancer - psychosocial
- mental health
- education
- employment
- health insurance
- chronic symptoms
- physical/body image
