Paediatric Oncology Flashcards
what is cancer and its features?
- Abnormal cells dividing in an uncontrolled way
- Gene changes
- Stimulates own blood supply
- Local invasion
- Metastatic spread via blood or lymphatic systems
how common are cancers in childhood?
- Rare -1821 cases < 15 in UK (per year 2013-15)
- Scotland 130 / yr
- 1/500 pre age 14
- GP sees 1 in career
- 10-15 per DGH catchment/year
- <1% all cancers
- Boys slightly more than girls
In the current era over 85% of childhood cancers are cured
are are paediatric 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
Classify childhood cancer based on the cell of origin, which is different to adult tumours where they focus on the primary site
why do children get caners?
Most childhood tumours are sporadic so bad luck. In about 10% there may be a cause. This can be due to a genetic syndrome the environment or a previous treatment:
- Genes - Down, Fanconi, BWS, Li-Fraumeni Familial Cancer Syndrome, Neurofibromatosis
- Environment - Radiation, Infection
- Iatrogenic - Chemotherapy, Radiotherapy
Summary:
- Childhood cancer is rare but does happen
- Usually sporadic
- Need to think of it
what is the diagnostic journey of a child with cancer?
Who to be worried about?
- Immediate referral - unexplained petechiae, hepatosplenomegaly
- Urgent referral:
- repeat attendance, same problem, no clear diagnosis
- new neuro symptoms, abdo mass
•Refer (to doctor or for urgent investigation):
- rest pain, back pain and unexplained lump
- lymphadenopathy ………
what are the symptoms of a brain tumour in different children ages?
whata re some oncological emergencies?
- Sepsis / febrile neutropenia
- Raised ICP
- Spinal cord compression
- Mediastinal mass
- Tumour lysis syndrome
Sepsis/febrile neutropenia:
Infection is major cause of morbidity/mortality
what are the risks and what organisms may be responsible?
•Risks:
- ANC < 0.5 x 109
- Indwelling catheter
- Mucosal inflammation
- High dose chemo/SCT
- Pseudomonas aeruginosa
- Enterobacteriaciae eg E coli, Klebsiella
- Streptococcus pneumoniae
- Enterococci
- Staphylococcus
- Fungi eg. Candida, Aspergillus
how does sepsis present?
- Fever (or low temp)
- Rigors
- Drowsiness
- Shock - Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis
how do you manage sepsis?
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
ABC - Oxygen and Fluids
Broad spectrum antibiotics
Inotropes
PICU
what are the early and late signs of ICP?
HC = head circumfrance
how would you investigate increased ICP?
- Imaging is mandatory (if safe)
- CT is good for screening
- MRI is best for more accurate diagnosis
Children often need anaesthetic to get good quality imaging and don’t want to do this if worried if unsafe due to risk of death or coning
how should raised ICP be 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
Spinal Cord Compression:
•Potential complication of nearly all paediatric malignancies - Affects 5 % of all children with cancer
what is the pathological process?
- Invasion from paravertebral disease via intervertebral foramina (40 % extradural)
- Vertebral body compression (30 %)
- CSF seeding (20 % intradural, extraspinal)
- Direct invasion (10 % intraspinal)
how would someone present with spinal cord compression?
- Symptoms vary with level:
- weakness (90 %)
- pain (55-95 %)
- sensory (10-55%)
- sphincter disturbance (10-35%)
how should psinal cord compression be 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
- Outcome depends on severity of impairment rather than duration between symptoms and diagnosis
- Mild impairment > 90 % recovery
- Paraplegic 65 % recovery
SVC syndrome or SMS: Superior vena cava syndrome, Superior mediastinal syndrome
- Rare <1 % of new paediatric malignancies
- Common causes - Lymphoma,Other – neuroblastoma, germ cell tumour, thrombosis
how does it present?
- SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS
- SMS: dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea
SVC syndrome or SMS - how should it be investigated?
- CXR / CT chest (if able to tolerate)
- Echo
Left is mediastinal
what is the management of SVC syndrome or SMS?
what is Tumour Lysis Syndrome?
- Metabolic derangement
- Rapid death of Tumour Cells
- Release of intracellular contents
- At or shortly after presentation
- Secondary to treatment
- (rarely spontaneous)
Tumor lysis syndrome is a group of metabolic abnormalities that can occur as a complication during the treatment of cancer, where large amounts of tumor cells are killed off (lysed) at the same time by the treatment, releasing their contents into the bloodstream
what are the clinical features of tumour lysis syndrome?
how do you treat tumour lysis syndrome?
What do we (oncologists) need to find out?
What it is and what harm is it causing?
- Scans (MRI scans are preferred as lack of radiation)
- Biopsy / pathology
- Cytogenetics
- Tumour markers
what is staging?
Where it is?
•Staging eg scans, bone marrow
Want to know if it has spread anywhere else using blood or lymphatic system
how should the treatment of cancers be carried out>
- Multimodal therapy based on specific disease and extent (plus patient factors)
- MDT approach
- National / international collaboration
- Clinical research
what are the different forms of treatment that can be used?
Surgery
Chemotherapy
Radiotherapy
Immunotherapy
Bone marrow transplant
New targeted agents
What are the risks of treatment? (chemotherapy and radiotherapy - acute and chronic)
what may the later effects of a cancer be?
Summary:
- Cancer in children is often _______
- Multimodal ___ approach – comes at a cost
- 1:700 of the adult population are childhood cancer survivors
- Survivors have increasing cumulative toxicity
curable
MDT
