Oncology Flashcards
How common is paediatric cancer?
<1% of all cancers
In which gender is childhood cancer more common?
Slightly more common in boys
When is the highest incidence of childhood cancer?
0-4y
What malignancies are most common in childhood?
Leukaemias (33%)
Brain tumours (25%)
40% are extracranial solid tumours
How does childhood cancer differ in its aetiology from adult cancer?
Not so much to do with wear and tear
More sporadic
What is the overall 5ys for childhood cancers?
> 80%
How are children’s cancers classified?
International classification of childhood cancer (ICCC)
Based on morphology and primary site
What are the aetiologies of childhood cancer?
Genes: down, Fanconis, BWS, Li-fraumeni familial cancer syndrome, neurofibromatosis
Environment: radiation (UV –> skin cancer), infections (e.g. HPV, EBV)
Iatrogenic: chemo/radio
What does Down syndrome increase the risk of?
ALL/AML (which may can be congenital)
What is Fanconi’s anaemia?
AD genetic condition assoc with short stature, developmental abnormalities, chromosomal fragility –> more likely to get adult cancers at younger age
What is BWS?
Beck-Weideman syndrome
Congenital disorder of growth with predisposition to tumour formation (get omphalocele, neonatal hypoglycaemia, macroglossia)
What is Li-Fraumeni familial cancer syndrome?
AD mutation of p53 gene (get sarcomas, leukaemias etc.)
What is neurofibromatosis?
Condition in which tumours grow in the nervous system (type I - café au lait spots, type 2 - vestibular schwannomas)
What things delay diagnosis of cancer?
Onset of symptoms after biological onset of disease
Symptoms (pressure/organ dysfunction)
Seeking of medical attention
Doctor recognising cancer as a possibility
Ix and diagnosis
What things merit immediate referral?
Unexplained petechiae, hepatosplenomegaly –> Ix for leukaemia/lymphoma that day
What things merit urgent referral?
Repeat attendance, same problem, no clear diagnosis, new neuro symptoms/abdo mass
See specialist within 48h
What things may get a normal referral within 2 weeks?
Rest pain, back pain, unexplained lump, lymphadenopathy
What programme has increased awareness of brain tumours in children?
Head smart campaign
has posters with symptoms - persistent/recurrent & neurological symptoms are ones to be aware of
What investigations can you do in paediatric oncology?
Scans, biopsy/pathology, tumour markers
Staging - scans, bone marrow
What are the common places for spread of cancer?
Bone, liver, lung, brain
What is involved in the treatment of childhood cancer?
Chemo, radio, surgery, immunotherapy
What are the acute side effects of chemo?
Hair loss, NV, diarrhoea/constipation, bone marrow suppression (anaemia, bleeding, infections)
What are the chronic side effects of chemo?
Organ impairment (heart, kidneys, eyes, nerves), reduced fertility, second cancer
What are the acute side effects of radiotherapy?
Skin irritation, lethargy, swelling, organ inflammation (lungs, bowel)
What are the chronic side effects of radiotherapy?
Fibrosis/scarring, second cancer, reduced fertility
What are the serious oncological emergencies?
Sepsis/febrile neutropenia Raised ICP Spinal cord compression Mediastinal mass Tumour lysis syndrome
What is the biggest cause of mortality/morbidity in paediatric oncology?
Sepsis/febrile neutropenia
What things put oncology patients at greater risk of sepsis/febrile neutropenia?
ANC <0.5x10^9, indwelling catheter, mucositis, high dose chemo/SCT
What opportunistic pathogens may cause sepsis/febrile neutropenia in cancer patients?
Pseudomonas aerginosa, eneterobacteriaeciae, e.g. E. coli, klebsiella, strep pneumoniae, eneterococci, staphylococcus, fungi, esp candida & aspergillus
What is the presentation of febrile neutropenia/sepsis?
Fever/hypothermia, rigors, drowsiness, shock (tachycardia, tachyopnoea, hypotension, prolonged cap refill, reduced UO, metabolic acidosis)
How do you investigate sepsis/febrile neutropenia?
IV access, blood culture, FBC, coag, UE, LFTs, CRP, lactate, CXR, other (urine microscopy, culture, throat swab, sputum culture/BAL, LP, viral PCRs, CT, USS
How do you manage febrile neutropenia/sepsis?
Oxygen, fluids, broad spectrum antibiotics, inotropes, PICU
In what age will you not see raised ICP?
Before 9-12m as fontantelles not fully closed yet
As pressure goes up what goes down and causes problems in RICP?
BP does down
What are the early signs of RICP?
Early morning headache/vomiting, tense fontanelle, increased head circumference
What are the late signs of RICP?
Constant headache, papilloedema, diplopia (VI palsy), loss of upgaze (if tumour near pineal gland), neck stiffness, status epilepticus, reduced CGD, cushing’s triad
What is cushing’s triad?
Low HR, high BP, low RR
Indicative of raised intracranial pressure
How do you Mx RICP?
Dexamethasone if due to tumour (reduces oedema/increases CSF flow), neurosurgery (urgent CSF diversion - ventriculostomy, EVD, VP shunt)
What is a ventriculostomy?
Hole in membrane of base of 3rd ventricle with endoscope
How long can you use an EVD for?
Only temporarily
Spinal cord compression is a potential complication of nearly all paediatric malignancies, but in which ones is it most common?
Ewings sarcoma (esp. in vertebral bodies), tumours in abdomen, germ cell tumours, neuroblastomas, medulloblastomas
What are the pathological processes in cancer that lead to cord compression?
Invasion from paravertebral disease via intervertebral foramina
Veretebral body compression
CSF seeding
Direct invasion
What sort of presentation do you get with spinal cord compression?
Weakness, pain, sensory, sphincter disturbance
How do you investigate spinal cord compression?
Urgent MRI
How do you treat spinal cord compression?
Start dexamethasone urgently to reduce peri-tumour oedema, definitive Rx with chemo when rapid response expected (or radio/surgery)
What does outcome of cord compression depend on?
Severity of impairment
What can mediastinal masses cause?
SVCS (superior vena cava syndrome) or SMS (superior mediastinal syndrome - obstruction of airway)
What can commonly cause SMS/SVCS?
Lymphoma
Neuroblastoma, germ cell tumours, thrombosis of the vena cava due to tumour compression
What does SVCS present like?
Facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS
What does SMS present like?
Dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea
How do you investigate suspected SMS/SVCS?
CXR/CT chest
Echo
How do you manage SMS/SCVS?
Keep upright & calm Urgent biopsy FBC, BM, pleural aspirate, GCT markers Chemo (may need presumptive Rx in absence of definitive histological diagnosis - steroids) Radio Rarely surgery if insensitive CVAD assoc. thrombosis - thrombolytics
What is tumour lysis syndrome?
Cancer cells grow so quickly, they can’t sustain themselves and start to die off (or lots of chemo –> lots of death at one time) –> release of intracellular content into plasma –> high levels K and phosphate –> flood kidneys and can’t be cleared –> arrhythmias
What are the clinical features of tumour lysis syndrome?
High potassium, urate, phosphate, low calcium
Acute renal failure - urate load, CaPO4 deposition in renal tubules
What is the treatment for tumour lysis syndrome?
Avoidance, ECG monitoring Hyperhydrate - 2.5ml/m2 QDS electrolytes Diuresis Never give potassium Decreas uric acid (allopurinol/urate oxidase/uricozyme) Treat hyperK - Ca resonium, salbutamol, insulin Renal replacement therapy (dialysis)