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
What is the epidemiology of childhood cancer?
- Rare in <15
- Scotland 130 per year
- 1 in 500 <14 year olds
- <1% of all cancer cases
- M>F (slightly)
What types of malignancies are seen in paediatrics?
- 33% Leukaemias
- 25% brain tumours
- 40% are extracranial solid tumours
What is the 5 year survival for childhood cancer?
80%
What is the basis of most childhood cancers?
Sporadic (some genetic basis)
How are childhood cancers classified?
- International Classification of Childhood Cancer (ICCC)
- Based on tumour morphology and (primary site)
- Standard classification is essential for comparing incidence and survival across regions and over time periods
What are the most common types of cancer in children?
- Leukaemia
- CNS tumours
- Lymphoma
- Soft tissue tumours
- Neuroblastoma
- Renal tumours
- Malignant bone tumours
- Retinoblastoma
- Germ cell tumours
- Hepatic tumours
When are the peaks of childhood cancer?
- Aged 0-4
- Adolescence
What are the causes of cancer in children?
Genetic: Down, Fanconi, BWS, Li-Fraumeni familial cancer syndrome
-Neurofibromatosis
Environment: radiation and infection
Iatrogenic: chemotherapy and radiotherapy induced
What are the steps in the diagnostic journey of childhood cancer?
- Biological onset of disease
- Symptom onset
- Seek medical attention
- Doctor recognises cancer as a possibility
- Investigation, diagnosis and treatment
When should a child be immediately referred to oncology?
- Unexplained petechiae
- Hepatosplenomegaly
When should a child have an urgent referral to oncology
- Repeat attendance with the same problem and no clear diagnosis
- New neuro symptoms or abdominal mass
When should a child be referred to oncology by phone call?
- Rest pain, back pain and unexplained lump
- Lymphadenopathy
What are the 5 most common signs of cancer in young people?
- Pain
- A lump, bump or swelling
- Extreme tiredness
- Significant weight loss
- Changes in a mole
What do oncologists need to find out?
What it is
- Scans (MRI+US)
- Biopsy and pathology
- Tumour markers
Where it is
-Staging and scans, bone marrow
How is cancer treated in children?
- Multimodal therapy based on specific disease and extent (plus patient factors)
- MDT approach
- Chemotherapy
- Surgery
- Radiotherapy
What are the acute risks of chemotherapy?
- Hair loss
- Nausea & vomiting
- Mucositis
- Diarrhoea / constipation
- Bone marrow suppression – anaemia, bleeding, infection
What are the chronic risks of chemotherapy?
- Organ impairment – kidneys, heart, nerves, ears
- Reduced fertility
- Second cancer
What are the acute risks of radiotherapy?
- Lethargy
- Skin irritation
- Swelling
- Organ inflammation – bowel, lungs
What are the chronic risks of radiotherapy?
- Fibrosis/scarring
- Second cancer
- Reduced fertility
What oncological emergencies are there?
- Sepsis / febrile neutropenia
- Raised ICP
- Spinal cord compression
- Mediastinal mass (SVCS/SMS)
- Tumour lysis syndrome
What is a major cause of mortality/morbidity in childhood cancer?
Infection
What are the risk factors for sepsis/ febrile neutropenia?
- ANC < 0.5 x 10^9
- Indwelling catheter
- Mucosal inflammation
- High dose chemo / SCT
What organisms can be implicated in sepsis/febrile neutropenia?
- Pseudomonas aeruginosa
- Enterobacteriaciae eg E coli, Klebsiella
- Streptococcus pneumoniae
- Enterococci
- Staphylococcus
- Fungi eg. Candida, Aspergillus
How does sepsis/febrile neutropenia present?
- Fever (or low temperature)
- Rigors
- Drowsiness
- Shock (tachycardia, tachypnoea, prolonged cap refill, reduced UO, metabolic acidosis)
How is sepsis/febrile neutropenia investigated?
- Blood culture, FBC, coag, UE, LFTs, CRP, lactact
- CXR
- Urine microscopy / culture
- Throat swab
- Sputum culture / BAL
- LP
- Viral PCRs
- CT / USS
How is sepsis/febrile neutropenia managed?
- ABC approach
- IV access
- Oxygen
- Fluids
- Broad spectrum antibiotics
- Inotropes
- PICU
What is the early presentation of raised ICP?
- Early morning headache/vomiting
- Tense fontanelle
- Increasing HC
What is the late presentation of raised ICP?
- Constant headache
- Papilloedema
- Diplopia (VI palsy)
- Loss of upgaze
- Neck stiffness
- Status epilepticus,
- Reduced GCS
- Cushings triad (low HR, high BP)
How is raised ICP investigated?
Imaging mandatory (if safe)
- CT good for screening
- MRI best for more accurate diagnosis
How is raised ICP managed?
Dexamethasone if due to tumour
- Reduce oedema and increase CSF flow
- 250 micro/kg IV STAT then 125 microg/kg BD
Neurosurgery - urgent CSF diversion
- Ventriculostomy – hole in membrane at base of 3rd ventricle with endoscope
- EVD (temporary)
- VP shunt
What cancers is spinal cord compression associated with?
Can complicate any paediatric malignancy
- Affects 5 % of all children with cancer
- 10-20 % Ewing’s or Medulloblastoma
- 5-10 % Neuroblastoma & Germ cell tumour
What is the 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)
How can spinal cord compression present?
Symptoms vary with level
- Weakness (90 %)
- Pain (55-95 %)
- Sensory (10-55%)
- Sphincter disturbance (10-35%)
How is spinal cord compression managed?
- Urgent MRI
- Start dexamethasone urgently to reduce peri-tumour oedema
- Definitive treatment with chemotherapy is appropriate when rapid response is expected (Surgery or radiotherapy are other options)
What does outcome of spinal cord compression depend on?
Outcome depends on severity of impairment rather than duration between symptoms and diagnosis
- Mild impairment > 90 % recovery
- Paraplegic 65 % recovery
What paediatric malignancies is SVC/SMS syndrome associated with?
- Rare <1 % of new paediatric malignancies
- Common causes include Lymphoma and other: neuroblastoma, germ cell tumour, thrombosis
How does SVC syndrome present?
- Facial, neck and upper thoracic plethora
- Oedema
- Cyanosis
- Distended veins
- Unwell
- Anxious
- Reduced GCS
How does SMS present?
- Dyspnoea
- Tachypnoea
- Cough
- Wheeze
- Stridor
- Orthopnoea
How should SVCS/SMS be investigated?
- CXR
- CT chest
- Echo
How should SVCS/SMS be managed?
-Keep upright & calm
-Urgent biopsy (ideally)
-Look to obtain important diagnostic information without GA
FBC, BM, pleural aspirate, GCT markers
-Definitive treatment is required urgently
-Chemotherapy is usually rapidly effective
-Presumptive treatment may be needed in the absence of a definitive histological diagnosis (steroids)
-Radiotherapy is effective
-May cause initial increased respiratory distress (surgery if insensitive)
What is the pathogenesis of tumour lysis syndrome?
- Metabolic derangement
- Rapid death of Tumour Cells
- Release of intracellular contents
- At or shortly after presentation
- Secondary to treatment
What are the clinical features of tumour lysis syndrome?
- Increased potassium
- Increase urate, relatively insoluble
- Increase phosphate
- Decreased calcium
- AKI (urate load and CaPO4 deposition in renal tubules)
What is the treatment for tumour lysis syndrome?
- Avoidance
- ECG Monitoring
- Hyperhydrate-2.5l/m2 -QDS electrolytes
- Diuresis
- Decrease uric acid
- Treat hyperkalaemia
- Renal replacement therapy
How can uric acid be decreased in tumour lysis syndrome?
- Urate oxidase uricozyme (rasburicase)
- Allopurinol
How is hyperkaelamia treated in tumour lysis syndrome?
- Ca resonium
- Salbutamol
- Insulin
What should never be given in tumour lysis syndrome?
POTASSIUM
