The Diagnosis of Brain Tumours in Children: A Guideline for Healthcare Professionals

Overview

This Guidelines summary covers the presenting symptoms and diagnosis of brain tumours in children, and when to refer for specialist management.

Referral from Primary Care

• High risk of tumour—SAME DAY referral to secondary care
• Lower risk^[A] —specialist assessment within 2 weeks 

Imaging

• High risk of tumour—URGENT CNS imaging
• Lower risk^[A] —CNS imaging within 4 weeks

Consider a Brain Tumour in Any Child Presenting With:

• Headache 
• Nausea and/or vomiting 
• Visual signs and symptoms: 
  • reduced visual acuity and/or fields 
  • abnormal eye movements 
  • abnormal fundoscopy 
• Motor symptoms and signs:
  • abnormal gait
  • abnormal co-ordination
  • focal motor weakness 
• Growth and endocrine symptoms:
  • growth failure (weight/height)
  • delayed, arrested or precocious puberty
  • galactorrhoea 
  • primary/secondary amenorrhea 
• Increasing head circumference 
• Behavioural change 
• Diabetes insipidus 
• Seizures (see www.nice.org.uk/guidance/qs27) 
• Altered consciousness (see www.rcpch.ac.uk/system/files/protected/page/Decon%20guidelines.pdf) 

Assess These Children With

• History: associated symptoms, any predisposing factors 
• Examination of: 
  • visual system 
  • motor system 
  • height and weight 
  • head circumference (<2 years of age) 
  • pubertal status 

Ask About Common Predisposing Factors

• Personal or FH of brain tumour, sarcoma, leukaemia, or early onset breast cancer 
• Neurofibromatosis 
• Tuberous sclerosis 
• Other familial genetic syndromes 

Assessment Pitfalls

• Initial symptoms of brain tumour can mimic other common illnesses 
• Symptoms frequently fluctuate—resolution then recurrence does not exclude a brain tumour 
• A normal neurological examination does not exclude a brain tumour 
• Language difficulties—use interpreter 

Headaches

• Consider a brain tumour in any child with a new, persistent^[B] headache 
• Headache in isolation, unlikely to be a brain tumour 
• Brain tumour headaches occur at any time of day 
• Children aged younger than 4 years may not be able to describe a headache—observe behaviour 

CNS Imaging Required With:

• Persistent headache that wakes a child from sleep 
• Persistent headache that occurs on waking 
• Persistent headache in a child under 4 years of age
• Confusion or disorientation with a headache 
• Persistent headache with 1 or more other symptoms 

Common Pitfalls

• Failure to reassess a child with a migraine or tension headache when the headache character changes 

Nausea and/or Vomiting

• Consider a brain tumour in any child with persistent^[C] nausea and/or vomiting 
• Head circumference should be measured and plotted in children under 2 years of age with persistent vomiting 

CNS Imaging Required With:

• Persistent vomiting on waking (NB: exclude pregnancy where appropriate) 
• Persistent nausea/vomiting with one or more other symptom 

Common Pitfalls

• Failing to consider a CNS cause for persistent nausea and vomiting 

Visual Signs and Symptoms

• Consider a brain tumour in any child with persistent^[D] visual abnormality 
• Visual assessment requires assessment of: 
  • visual acuity 
  • eye movements 
  • pupil responses 
  • optic disc appearance 
  • visual fields (≥ 5 years of age) 
• Pre-school and unco-operative children should be assessed by hospital eye service within 2 weeks of referral 
• Parent concern alone warrants referral for visual assessment 

CNS Imaging Required With:

• Papilloedema
• Optic atrophy
• New onset nystagmus 
• Reduction in visual acuity not due to refractive error 
• Visual field reduction
• Proptosis
• New onset paralytic squint
• Visual symptom with 1 or more other symptom

Common Pitfalls

• Failure to fully assess vision—REFER IF NECESSARY
• Failure of communication between community optometry and primary and secondary care

Head Circumference

• Consider a brain tumour in any child under two years with an increasing head circumference outside the normal range in comparison to their height and weight 
• Careful assessment of other signs and symptoms of a brain tumour should be undertaken in these babies 

CNS Imaging Required With:

• Rapid rate of head circumference growth crossing centiles 
• Increasing head circumference with any other associated symptoms 

Common Pitfalls

• Failing to measure and monitor head circumference in a baby or young child with persistent vomiting 

Motor Symptoms and Signs

• Consider a brain tumour in any child with persisting^[E] motor abnormality 
• Motor assessment requires history or observation of: 
  • sitting and crawling in infants 
  • walking and running 
  • handling of small objects 
  • handwriting in school age children 
• Brain tumours can cause a loss or change in motor skills and this can be subtle e.g. ability to play computer games 

CNS Imaging Required With:

• Regression in motor skills 
• Focal motor weakness 
• Abnormal gait/co-ordination (unless local cause) 
• Bells palsy with NO improvement within 4 weeks 
• Swallowing difficulties (unless local cause) 
• Head tilt/torticollis (unless local cause) 
• Motor symptom with one or more other symptom 

Common Pitfalls

• Attributing abnormal gait/balance to middle ear disease with no corroborating findings 
• Failure to identify swallowing difficulties and aspiration as a cause of recurrent chest infections 

Growth and Endocrine

• Consider a brain tumour in any child with any combination of growth failure, delayed/arrested puberty and polyuria/polydipsia 
• Early specialist assessment if required for:
  • precocious puberty/delayed or arrested puberty
  • growth failure
  • galactorrhoea
  • primary or secondary amenorrhoea

CNS Imaging Required With:

• Growth or endocrine symptom with 1 or more other symptoms

Common Pitfalls

• Failing to consider a CNS cause in children with weight loss and vomiting
• Failure to consider diabetes insipidus in children with polyuria and polydipsia

Behaviour

• Consider a brain tumour in any child with new onset lethargy, mood disturbance, withdrawal or disinhibition 

Common Pitfalls

• Failing to consider a physical cause for behavioural symptoms

Presenting Symptoms of Brain Tumours by Sub-specialty

Psychiatry

• Anorexia   
• Behavioural change   
• Depression 
• Psychosis  

Ophthalmology

• Papilloedema 
• Decreased visual acuity 
• Nystagmus/Parinauds 
• Diplopia 
• Squint 
• Visual field defect 
• Blindness 
• Ptosis 
• Proptosis 
• Ocular palsies 
• Ophthalmoplegia 

Ear, Nose, and Throat

• Dizziness  
• Vertigo  
• Torticollis  
• Head tilt  
• Hearing loss  
• Tinnitus

Community Paediatrics

• Developmental delay  
• Developmental regression  
• Increasing head circumference  

Gastroenterology

• Nausea and vomiting  
• Abdominal pain  
• Reflux  
• Failure to thrive  
• Dysphagia  

Neurology

• Seizures  
• Motor weakness  
• CN palsies  
• Ataxia/cerebellar  
• Focal neurological deficits  

Endocrinology

• Growth problem 
• Hypo-pit/pituitary dysfunction 
• Diabetes insipidus 
• Precocious or delayed puberty 
• Menstrual irregularities 
• Galactorrhoea 
• Gynaecomastia 
• Cushing’s 
• Obesity/weight gain 

Respiratory

• Recurrent chest infections  
• Apnoeas   

Footnotes

[A] Lower risk=CNS tumour in differential diagnosis, low index of suspicion

[B] Persistent=continuous or recurrent headache present for more than 4 weeks

[C] Persistent=nausea and/or vomiting present for more than 2 weeks  

[D] Persistent=visual abnormality present for more than 2 weeks 

[E] Persistent=motor abnormality present for more than 2 weeks