Paediatric Oncology Flashcards
How common is children’s cancer?
Rare
Types of paed malignancy
33% leukaemia
25% brain tumours
40% extracranial solid tumours
Is child cancer biologically different from adult cancer?
Yes
How do children get cancer?
Mostly sporadic
Occasionally genetic basis
What is the 5 year survival for childhood cancer?
80%
What is the classification of child cancer based on?
Tumour morphology
Primary site
What organisation is used to classify childhood cancer?
Internal classification of childhood cancer (ICCC)
What is the most common age to get childhood cancer?
0 - 4 y/o
Causes of Childhood cancer
Genes - Downs - Fanconi - BWS - Li-framumeni familial cancer syndrome - Neurofibromatosis Environmental - radiation - infection - EBV, HPV Iatrogenic - chemo - radiotherapy
What cancer is downs associated with?
Leukaemia
What cancers are Fanconi associated with?
Anal / vulval cancers - adult type
Anaemia and developing leukaemia
What cancers are BWS associated with?
Hepatoblastomas
What cancers are Li-franumeni familial cancer syndromes associated with?
Leukaemia
Sarcomas
Brain tumours
What cancers are neurofibromatosis associated with?
Neurofibromas
Sarcomas
Leukaemias
Who needs immediate referral?
Unexplained petechiae
Hepatosplenomegaly
Who needs urgent referral within 48 hours?
Repeated attendance, same problem, no clear diagnosis New neuro symptoms Abdominal mass Rest pain Back pain Unexplained lump Lymphadenopathy
Possible symptoms of childhood cancer
Unable to urinate / blood in urine Lumps Swollen glands Persistent back pain Persistent headaches Frequent bruising Persistent unexplained tiredness Unexplained seizures or changes in vision or behaviour Persistent abdo pain /swelling unexplained vomiting Unexplained sweating or fever unexplained weight loss or low apetite Changes in appearance of the eyes or unusual eye reflections in photos Frequent infections / flu like symptoms
Brain tumour symptoms in children
Persistent / recurrent vomiting
Persistent / recurrent headache
Abnormal balance / walking/ coordination
Blurred or double vision
Abnormal eye movements
Behaviour change
Lethargy
Fits / seizures (not with a fever in < 5s)
Abnormal head position
Delayed or arrested puberty in young people
Teenage cancer symptoms
Unexplained Persistent Pain Lump / bump / swelling Extreme tiredness Significant weight loss Changes in a mole
How to find out what cancer it is
Scans
Biopsy / pathology
Tumour markers
How to find out where the cancer is
Staging e.g. scans, bone marrow
Treatment of childhood cancer
Chemotherapy
Surgery
Radiotherapy
Risks of radiotherapy
Acute - lethargy - skin irritation - swelling - organ inflammation (bowel / lungs) Chronic - fibrosis / scarring - second cancer - reduced fertility
Risks of chemo
Acute - hair loss - nausea and vomiting - mucositis - diarrhoea / constipation - bone marrow suppression (anaemia, bleeding, infection) Chronic - organ impairment (kidneys, hearts, nerves, ears) - reduced fertility - second cancer
What are examples of oncological emergencies?
Sepsis / febrile neutropenia Raised ICP Spinal cord compression Mediastinal mass Tumour lysis syndrome
Risks of sepsis / febrile neutropenia
ANC < 0.5x10 9
indwelling catheter
mucosal inflammation
high dose chemo / SCT
Causative organisms of febrile neutropenia / sepsis
Psueodmonas aeruginosa
Strep pneumoniae
Enterococci
Fungi e.g. Candida
Presentations of sepsis / febrile neutropenia in paed oncology
Fever / low temp
Rigors
Drowsiness
Shock
Presentation of septic shock
Tachycardia Tachypnoea Hypotension Prolonged cap refill time reduced UO Metabolic acidosis
Investigations of febrile neutropenia / sepsis
IV access Blood culture / FBC / U and Es, coagulation / LFTs, CRP, lactate CXR urine microscopy / culture Throat swab Sputum culture / BAL LP viral PCRs CT / USS
Treatment of sepsis / febrile neutropenia
ABC - oxygen - fluids Broad spectrum Ax Inotropes PICU
When is raised ICP uncommon?
In children < 1 y/o due to fontanelles
Presentation of raised ICP
Early morning headache / vomiting Tense fontanelle Increasing head circumference disproportionate to body Late - constant headache - papilloedema - diplopia - loss of up gaze - neck stiffness - status elipticus - reduced GCS - cushings triad (low HR, high BP)
Investigations for raised ICP
CT
MRI (more accurate)
Treatment of raised ICP
If due to tumour = dexamethasone For urgent CSF diversion = neurosurgery - ventriculostomy - EVD (temporary) - VD shunt
What is spinal cord compression a potential malignancy of?
nearly all paediatric malignancies
Pathological processes of spinal cord compression in children
Invasion of paravertebral disease via intervertebral foramina (40% extradural) Vertebral body compression (30%) CSF seeding (20% intradural, extraspinal) Direct invasion (10% intraspinal)
Presentations of spinal cord compression
Symptoms vary with level
- weakness (90%)
- pain (55-95%)
- Sensory (10-55%)
- sphincter disturbance (10-35%)
Treatment of Spinal cord compression
Urgent MRI Dexamethasone urgently to reduce peri tumour oedema Chemotherapy Surgery Radiotherapy
Prognosis of spinal cord compression
Mild impairment > 90% recovery
Paraplegia 65% recovery
What does the prognosis of spinal cord compression depend on?
The severity of impairment
Common causes of SVC syndrome / SMS
lymphoma
neuroblastoma
germ cell tumour
thrombosis
Presentation of SVCS
facial, neck and upper thoracic plethora Oedema Cyanosis Distended veins ill anxious reduced GCS
Presentation of SMS
Dyspnoea Tachypnoea Cough Wheeze Stridor Orthopnoea
Investigations of SVC syndrome / SMS
CXR / CT chest ECHO Urgent biopsy FBC, BM, Pleural aspirate GCT markers
Treatment of SVC syndrome / SMS
Keep upright in position they are comfortable in and calm
Chemotherapy
Steroids may be needed in the absence of a histological diagnosis
Radiotherapy
Surgery - rarely if infinitive
Pathology of tumour lysis syndrome
Metabolic derangement
Rapid death of tumour cells
Release of intracellular contents
At or shortly after presentation
What is tumour lysis syndrome secondary to?
Treatment
Rarely spontaenous
Presentation of tumour lysis syndrome
Increased K Increased urate, relatively insoluble Increased P Decreased Ca Acute renal failure - urate load - CoPO4 deposition in renal tubules
Treatment of tumour lysis syndrome
Avoidance ECG monitoring Hyperhydrate QDS electrolytes Diuresis Decrease uric acid - allopurinol - urate oxidase Treat hyperkalaemia - Ca resonium - salbutamol - insulin Renal replacement therapy
What do you NEVER give in tumour lysis syndrome?
Potassium
What do you check the red reflex in babies for?
Retinoblastoma
Congenital cataract
Poor prognostic factors for ALL
Male sex Age < 2 or > 10 WBC > 20 x 10^9 at diagnosis T or B cell surface markers Non Caucasian
What is the most common ocular malignancy found in childhood?
Retinoblastoma
Inheritance of retinoblastoma and what % are inherited?
Autosomal dominant
10% inherited
Pathology of retinoblastoma
Loss of function of the retinoblastoma tumour suppressor gene on chromosome 13
Possible presentations of retinoblastoma
Absence of red reflex, replaced by a white pupil (leukocoria)
Strabismus
Visual problems
What is the most common presentation of retinoblastoma?
Absence of red reflex and replacement with a white pupil (leukocoria)
Treatment of retinoblastoma
Enucleation
External beam radiation therapy
Chemo
Photocoagulation
Prognosis of retinoblastoma
Excellent - > 90% survive into adulthood
