Brain and central nervous system (CNS) cancers

Scottish referral guidelines for suspected cancer
Centre for Sustainable Delivery

Emergency (same day) referral

Refer a person with two or more of the following as an emergency (same day):

  • New headache
  • New seizure
  • Papilloedema
  • New focal neurological deficit

 

Urgent suspicion of cancer (USC) referral

Refer a person with a headache where there is concern about a brain/CNS cancer plus one or more of the following features:

  • Cognitive change – symptomatic or noted by others
  • Personality change
  • History of cancer (especially lung, breast, melanoma or renal)
  • History of HIV

 

Assessment of suspected brain/CNS cancers

The anatomical location of brain and CNS cancers influences presenting features that include physical, cognitive and psychological components. Better safe than tumour contains more information on possible symptoms of brain cancer.

If there is concern that a person may have a brain/CNS cancer the following should be assessed for:

  • Headache suggestive of raised intracranial pressure (ache generalised over the cranium, worst on awakening, may awaken the person from sleep, aggravated by bending or stooping, severity gradually progresses, associated nausea/vomiting)
  • Papilloedema (optic nerve head swelling due to raised intracranial pressure) - if there is uncertainty, the person should be referred urgently to an optometrist for assessment
  • Focal neurological deficit
  • New seizures (will often require a witness account) including those without collapse (e.g. vacant episodes, transient self-limiting motor, or sensory change)
  • Changes in cognition or personality (symptomatic or noted by others)
  • Changes in speech such as difficulty finding words or using the wrong words
  • History of Human Immunodeficiency Virus (HIV) or cancer originating in other parts of the body (especially lung, breast, melanoma, or renal)

Single clinical features alone are poorly predictive of brain cancers e.g. headache without other clinical features has a PPV of 0.1%22. However, by the time of diagnosis, headache and cognitive symptoms co-occur with a PPV of 7.2%, supporting the importance of searching for “headache plus” other symptoms22. At the early stages of disease, cognitive changes may be subtle or go unnoticed by patients and may only be apparent retrospectively.

Good practice points

Referral guidance:

Boards' local pathways for referral of a headache which raises suspicion for brain cancer should be followed. If this includes direct access to imaging it must be delivered urgently to avoid a delay in diagnosis. Imaging should not be used in place of an emergency referral if that is more appropriate.

When referring to secondary care, a note should be made of any recent brain imaging as this may alter the urgency and the need for further radiology assessment.

Refer a person urgently to an optometrist for assessment if there is uncertainty about the presence of papilloedema or visual field loss. If papilloedema is confirmed, the consideration should be given to same day referral to secondary care. A clear plan should be made as to who will be responsible for the follow-up of the results of an optometry assessment.

 

Headache management:

Many people presenting with headache will not fit into the referral guideline above. CfSD has published the National headache pathway to guide referral in other scenarios.

 

Assessing changes in cognition:

Changes in cognition may not be volunteered by a person presenting with signs and symptoms of a brain tumour and direct enquiry may have to be made. The Semantic Verbal Fluency Test (SVFT) is a quick test which can be done easily in practice and may indicate cognitive deficit if the score is reduced (i.e. less than 17 different animals named in 1 minute). A headache concerning for a brain tumour along with a reduced SVFT score has been shown to have a PPV higher than 5%23. Please note that a SVFT score may be reduced in other conditions such as dementia, previous serious head injury, stroke, learning disabilities or for those whose first language is not English.

 

Background

There are around 1,000 incidences of brain and central nervous system (CNS) tumours in Scotland a year1. Approximately 50% of these are malignant brain cancers. Currently around 1 in 3 people (34% of adults aged 15-99) diagnosed with brain and CNS cancers in Scotland survive their disease for 1 year or more (data covering years of diagnosis 2015-2019)19. Brain cancer patients in Scotland are more likely than other groups of cancer patients to be diagnosed via an emergency route, with greater than 70% of brain and other CNS cancer patients being diagnosed through emergency routes1.

Timely diagnosis of brain and CNS cancers is challenging. Most patients with neurological symptoms are diagnosed with benign disease, meaning primary healthcare professionals encounter brain and CNS cancers infrequently20. Multiple consultations prior to referral can occur commonly among adults subsequently diagnosed with primary brain cancers. Evaluation of the National Cancer Patient Experience Surveys (CPES) (England) showed that 39% of brain and CNS cancer patients had three or more pre-referral consultations with a GP compared with an average of 25% for all cancers21.