Paediatric Oncology Flashcards
types of cancer in children
- Leukaemia – 33%
- CNS tumours – 25%
- Lymphomas – 10%
- Neuroblastoma – 7%
- Soft tissue sarcomas – 7%
- Wilm’s tumour – 6%
- Bone tumours – 4%
- Retinoblastoma – 3%
- Other – 7%
what is the overall 5 year survival for childhood cancer?
80%
genes implicated in childhood cancer
o Fanconi
o BWS
o Li-Fraumeni Familial Cancer Syndrome
o Neurofibromatosis
viral infections associated with childhood cancer
§ EBV • Burkitt, Hodgkin, nasopharyngeal § Hepatitis B • Hepatic carcinoma § HIV • Kaposi
iatrogenic causes of child cancer
o Radiotherapy § Craniospinal • CNS tumours § Chest • Breast o Chemotherapy § Etoposide • AML
what to be worried about in child potential cancer: immediate referral
unexplained petechiae
hepatosplenomegaly
what to be worried about in child potential cancer: urgent referral
repeat attendance, same problem, no clear diagnosis
new neuro symptoms, abdo mass
what to be worried about in child potential cancer: refer (to doctor or for urgent investigations)
rest pain
back pain
unexplained lump
lymphadenopathy
risks of cancer chemotherapy: acute
o Hair loss o Nausea & vomiting o Mucositis o Diarrhoea / constipation o Bone marrow suppression – anaemia, o bleeding, infection
risks of cancer chemotherapy: chronic
o Organ impairment – kidneys, heart,
o nerves, ears
o Reduced fertility
o Second cancer
risks of cancer radiotherapy: acute
o Lethargy
o Skin irritation
o Swelling
o Organ inflammation – bowel, lungs
risks of cancer radiotherapy: chronic
o Fibrosis / scarring
Figure 2 Headsmart: Be Brain Tumour Aware
o Second cancer
o Reduced fertility
childhood oncological emergencies
- Sepsis/febrile neutropenia
- Raised ICP
- Spinal cord compression
- Mediastinal mass
- Tumour lysis syndrome
febrile neutropenia in children: risks
ANC < 0.5x109
indwelling catheter
mucosal inflammation
high dose chemo/SCT
febrile neutropenia in children: bacteria
- Pseudomonas aeruginosa
- Enterobacteriaciae eg E coli, Klebsiella
- Streptococcus pneumoniae
- Enterococci
- Staphylococcus
- Fungi eg. Candida, Aspergillus
febrile neutropenia in children: presentation
fever (or low temp)
rigors
drowsiness
shock
febrile neutropenia in children: management
• IV access • Blood culture, FBC, coag, U+E, LFTs, CRP, lactate • CXR • Other o Urine microscopy / culture o Throat swab o Sputum culture / BAL o LP o Viral PCRs o CT / USS • ABC o Oxygen o Fluids • Broad spectrum antibiotics • Inotropes • PICU
define febrile neutropenia
• Neutrophils <0.5x109/l and
• Fever > 38.0 C (assessed by any means)
• In addition, patients for whom there is a clinical suspicion of sepsis in the absence of fever
should be treated as potentially bacteraemic e.g. unexplained abdominal pain or generally
unwell
raised ICP: presentation early
• Early o Early morning • Headache/vomiting o Tense fontanelle o Increasing HC
raised ICP: presentation late
o Constant headache o Papilloedema o Diplopia (VI palsy) o Loss of upgaze o Neck stiffness o Status epilepticus, o Reduced GCS o Cushings triad (low HR, high BP)
raised ICP: investigation
CT for screening
MRI for accutate diagnosis
raised ICP: managment
raised ICP:
spinal cord compression who gets it
• Potential complication of nearly all paediatric malignancies
• Affects 5 % of all children with cancer
o 10-20 % Ewing’s or Medulloblastoma
o 5-10 % Neuroblastoma & Germ cell tumour
o Diagnosis (65 %), relapse, progression
• Pathological processes
o Invasion from paravertebral disease via intervertebral foramina (40 % extradural)
o Vertebral body compression (30 %)
o CSF seeding (20 % intradural, extraspinal)
o Direct invasion (10 % intraspinal)
• Rare but devastating
• ~5% of solid tumours at presentation
• Much more common in terminal phase
• Dumb bell tumour
o NBL 27%
o Ewing’s 23%
o Rhabdo 15%
o Osteo 12%
spinal cord compression: clinical features
• Weakness 90% o Ambulatory 55% o Non-ambulatory 33% o Paraplegic 12-57% • Spine tenderness ~70% • Sphincter disturbance 51-57% • Sensory disturbance 14-52% • Gait disturbance ~10% • Back pain 61-80%
spinal cord compression: managment
• MRI • Dexamethasone 1mg/kg (max 16 mg) • Chemo • DXT • Surgery o Debulking o Laminectomy o Laminotomy o Laminoplasty • Bleeding • SVC obstruction • Airway obstruction • Leucostasis • VOD • Raised ICP • Bone marrow failure • Pancreatitis • Encephalopathy • Thrombosis • Refeeding syndrome
SVC syndrome: causes
lymphoma
other - neuroblastoma, germ cell tumour, thrombosis
SVC syndrome: presentation
o SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins,
ill, anxious, reduced GCS
o SMS: dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea
SVC syndrome: investigation
o CXR / CT chest (if able to tolerate)
o Echo
SVC syndrome: management
• Keep upright & calm
• Urgent biopsy (ideally)
• Look to obtain important diagnostic information without GA
o FBC, BM, pleural aspirate, GCT markers
• Definitive treatment is required urgently
o Chemotherapy is usually rapidly effective
o Presumptive treatment may be needed in the absence of a definitive histological
diagnosis (steroids)
o Radiotherapy is effective
o May cause initial increased respiratory distress
o Rarely surgery if insensitive
o CVAD-associated thrombosis should be treated by thrombolytic therapy
• Most of underlying malignancies have a good prognosis
tumour lysis syndrome: what is it
- Metabolic derangement
- Rapid death of tumour cells
- Release of intracellular contents
- At or shortly after presentation
- Secondary to treatment
- (rarely spontaneous)
tumour lysis syndrome: clinical features
• Increased potassium • Increased urate, relatively insoluble • Increased phosphate • Decreased calcium • Acute renal failure o Urate load o CaPO4 deposition in renal tubules
tumour lysis syndrome: treatment
• Treatment • ECG monitoring • Hyperhydrate 2.5l/m2 • Diuresis • Never give potassium • Treat hyperkalaemia o Ca Resonium o Salbutamol o Insulin • At least qds electrolytes • Watch urate and PO4 closely • Decrease uric acid o Urate oxidase-uricozyme (rasburicase) o Allopurinol • Renal replacement therapy
