Paediatric Oncology

Question 1

Whay is cancer?

Answer 1

Group of abnormal cells dividing in uncontrollable way:

• Usually occurs due to gene changes
• Stimulates own blood supply
• Can cause local invasion or metastatic spread via blood or lymphatic systems

Question 2

Cancer - aetiology

Answer 2

• Genes
  
  • Down
  • Fanconi
  • BWS
  • Li-Fraumeni familial cancer syndrome
  • Neurofibromatosis
• Environment
  
  • Radiation
  • Infection
• Iatrogenic
  
  • Chemotherapy
  • Radiotherapy

Question 3

Cancer - epidemiology

Answer 3

• Very rare
  
  • GP sees 1 in career
• Usually at younger age
  
  • Between 0-4

Question 4

What are the most common cancers in children?

Answer 4

1. Leukaemias
2. CNS tumours
3. Lymphomas

Question 5

How are cancers in children classified?

Answer 5

Classified by cell origin, not on primary site:

• International Classification of Childhood Cancer (ICCC)
• Based on tumour morphology (and primary site)

Question 6

Cancer - clinical features

Answer 6

• Unexplained petechiaea
• Hepatospenomegaly
• Neuro symptoms
• Abdominal mass
• Rest pain, back pain
• Unexplained lump
• Lymphadenopathy
• Unexplained vomiting
• Unexplained sweating or fever

Question 7

What are some oncological emergencies?

Answer 7

• Sepsis/febrile neutropenia
• Raised ICP
• Spinal cord compression
• Mediastinal mass
• Tumour lysis syndrome

Question 8

Sepsis/febrile neutropneia - risk factors

Answer 8

• ANC <0.5 x10⁹
• Indwelling catheter
• Mucosal inflammation
• High dose chemo/SCT

Question 9

Sepsis/febrile neutropneia - presentation

Answer 9

• Fever (or low temperature)
• Rigors
• Drowsiness
• Shock
  
  • Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis

Question 10

What are signs of shock?

Answer 10

• Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis

Question 11

Sepsis/febrile neutropenia - investigations

Answer 11

• Bloods
  
  • Culture, FBC, coagulation, U&Es, LFT, CRP, lactate
• CXR
• Other
  
  • Urine microscopy/culture
  • Throat swab
  • Sputum culture
  • LP
  • Viral PCRs
  • CT/USS

Question 12

Sepsis/febrile neutropenia - management

Answer 12

• ABC
  
  • Oxygen
  • Fluids
• Broad spectrum antibiotics
• Inotropes
• PICU

Question 13

Raised ICP - presentation

Answer 13

• Early
  
  • Early morning headache/vomiting
  • Tense fontanelle
  • Increasing head circumference
• Late
  
  • Constant headache
  • Papilloedema
  • Diplopia (VI palsy)
  • Loss of upgaze
  • Neck stiffness
  • Status epilepticus
  • Reduced GCS
  • Cushing triad (low HR, high BP)

Question 14

Raised ICP - investigations

Answer 14

• Imaging
  
  • CT good for screening
  • MRI best for accurate diagnosis

Question 15

Raised ICP - management

Answer 15

• Dexamethasone if due to tumour
  
  • Reduce oedema dn increase CSF flow
• Neurosurgery
  
  • Ventriculostomy
  • EVD (temporary)
  • VP shunt

Question 16

Spinal cord compression - pathophysiology

Answer 16

• Invasion from paravertebral disease via intervertebral foramina
• Vertebral body compression
• CSF seeding
• Direct invasion

Question 17

Spinal cord compression - presentation

Answer 17

• Varies depending on where is compressed
  
  • Weakness (90%)
  • Pain (60%)
  • Sensory
  • Sphincter disturbance

Question 18

Spinal cord compression - investigations

Answer 18

• MRI – urgent

Question 19

Spinal cord compression - management

Answer 19

• Dexamethasone
  
  • Urgently to reduce peri-tumour oedema
• Chemotherapy
  
  • Surgery or radiotherapy other definitive treatment options

Question 20

Spinal cord compression - prognosis

Answer 20

• Depends on severity of impairment rather than duration between symptoms and diagnosis

Question 21

SVC syndrome - aetiology

Answer 21

• Lymphoma
• Neuroblastoma
• Germ cell tumour
• Thrombosis

Question 22

SVCS - presentation

Answer 22

• SVCS
  
  • Facial, neck and upper thoracic plethora
  • Oedema
  • Cyanosis
  • Distended veins
  • Ill
  • Anxious
  • Reduced GCS
• SMS
  
  • Dyspnoea
  • Tachypnoea
  • Cough
  • Wheeze
  • Stridor
  • Orthopnoea

Question 23

SVC syndrome - investigations

Answer 23

• CXR/CT chest
• Echo
• Biopsy
• Bloods
  
  • FBC, GCT markers

Question 24

SVC syndrome - management

Answer 24

• Urgent biopsy
• Chemotherapy
• Radiotherapy
• Rarely surgery
• CVAD associated thrombosis
  
  • Thrombolytic therapy

Question 25

Tumour lysis syndrome - aetiology

Answer 25

* Secondary to treatment

Question 26

Tumour lysis syndrome - pathology

Answer 26

* Rapidly growing tumour starts to die * Releasing internal contents into blood stream

Question 27

Tumour lysis syndrome - clinical features

Answer 27

* Increased potassium * Increased urate * Increased phosphate * Decreased calcium * Acute renal failure * Urate overload * CaPO4 deposition in renal tubules

Question 28

How do the following change in tumour lysis syndrome: - K - urate - PO - Ca

Answer 28

* Increased potassium * Increased urate * Increased phosphate * Decreased calcium

Question 29

Tumour lysis syndrome - management

Answer 29

* Prophylaxis * Give fluids, monitor electrolytes * Diuresis * NEVER give potassium or phosphate when giving fluid * Decrease uric acid * Urate oxidase uricozyme (Rasburicase) * Allopurinol * Treat hyperkalaemia * Ca Resonium * Salbutamol * Insulin * Renal replacement therapy

Question 30

Paediatric cancer - investigations

Answer 30

* Scans * MRI * CT if unable to tolerate MRI * Biopsy/pathology * Cytogenetics * Tumour markers * Staging * Chest x-ray or CT chest * Bone scan

Question 31

Paediatric cancer - treatment

Answer 31

* Surgery * Chemotherapy * Radiotherapy * Immunotherapy * Bone marrow transplants * Newer drugs * Clinical trials, biologics

Question 32

What are the side effects of chemotherapy?

Answer 32

Question 33

What are the side effects of radiotherapy?

Answer 33