Top Tips for GPs holding a 2WW Conversation
Macmillan 2WW guidance
Scope:
- diagnosis of suspected brain tumours in adults age 18 years and older
- management, including:
- primary cerebral tumours
- secondary brain tumours, ie metastases from a known or unknown primary site in adults
- multidisciplinary assessment and primary management decisions for brain tumour following initial imaging
- further management of brain tumour following initial imaging and surgical biopsy or resection
Out of scope:
- specific management of:
- skull-based tumours, including acoustic neuromas
- optic gliomas
- medulloblastomas
- pituitary tumours
- central nervous system (CNS) lymphomas
- pineal tumours
- tumours in patients under age 18 years
Definition:
- tumour found in the intracranial portion of the CNS – includes [1]:
- tumours of the brain substance, many of which arise from glial or support cells (gliomas)
- tumours of the meninges
- metastases from other primary sites
- primary tumours are classified by the World Health Organization (WHO) from grade I (least aggressive) to grade IV (most aggressive) [1]
Incidence:
- brain cancer is amongst the top 20 most common cancers diagnosed each year in both men and women [7]
- account for approximately 2% of all cancer deaths [7]
- annual incidence of high grade glioma is approximately 5/100,000 [2]
- incidence is highest in adults age 60-80 years [7]
Risk factors [1]:
- while a number of epidemiological risk factors have been suggested, the aetiology of primary brain tumours is not well understood
- the only known associations are:
- family history of early-onset malignancy
- a variety of familial syndromes, eg:
- neurofibromatosis types 1 and 2
- tuberous sclerosis
- previous radiotherapy to the head or neck (rare)
- a weakened immune system (slightly increased risk)
Prognosis:
- in general, malignant CNS tumours have a poor prognosis [1]
- depends on:
- tumour grade and histology [2]
- anatomical site (whether the tumour is suitable for resection) [1]
- primary brain tumours only very rarely disseminate outside the CNS [2]
- other factors to note regarding prognosis include [1]:
- the rigid nature of the skull means that even a small, slow-growing tumour can cause severe symptoms and detrimental (even fatal) effects when it raises intracranial pressure (ICP)
- slow-growing tumours in the brain can infiltrate extensively into adjacent normal tissue, making excision impossible
- the vital functions of the brain, in which these tumours arise, pose a particular challenge for surgical excision
- a slow-growing tumour may undergo transformation to an aggressive tumour
- favourable prognostic factors for glioma [2]:
- lower tumour grade
- radical tumour resection
- younger age – age less than 50 years
- good performance status
- intact neurological function
The most common brain tumours are [1]:
- low-grade gliomas, which include [1]:
- astrocytomas
- oligodendrogliomas
- mixed oligoastrocytomas
- high-grade gliomas, which include [2]:
- anaplastic astrocytomas
- glioblastomas
- mixed anaplastic oligoastrocytomas
- anaplastic oligodendrogliomas
- meningiomas
- metastases
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Efforts are made to ensure the accuracy and agreement of these guidelines. However, we cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties
UHB Patient Information Leaflet
NBT Patient Information Leaflet
NSCCG Sign off form 2017
Updated in line with NICE Guideline NG12: Suspected cancer: recognition and referral to coincide with updated BNSSG 2WW Referral forms
Oncology NSCCG sign off form
Date of publication: 31-Jan-2014
This care map has been redrafted to provide a more condensed specialist overview of brain tumours and to include information from the following guidelines:
- [2] Stupp R, Tonn JC, Brada M et al. High-grade malignant glioma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2011; 21 Suppl 5: v190-v193.
- [4] The Royal College of Physicians (RCP) and The Royal College of Radiologists (RCR). Evidence-based indications for the use of PET-CT in the United Kingdom 2012. London: RCP and RCR; 2012.
- [6] National Institute for Health and Clinical Excellence (NICE). Referral guidelines for suspected cancer: CG27. London: NICE; 2005.
- [7] Department of Health (DH). Direct access to diagnostic tests for cancer: best practice referral pathways for general practitioners. London: DH; 2012.
- [9] National Comprehensive Cancer Network (NCCN). Central nervous system cancers. Version 2.2013. Fort Washington, PA: NCCN; 2013.
- [11] Driver and Vehicle Licensing Agency (DVLA). At a glance guide to the current medical standards of fitness to drive. Swansea: DVLA; 2013.
A literature search identified an update to the following, but no changes to clinical recommendations were required:
- [5] Clinical Knowledge Summaries (CKS). Brain tumour - suspected. Newcastle upon Tyne: CKS; 2012.
- [8] Patil CG, Pricola K, Sarmiento JM et al. Whole brain radiation therapy (WBRT) alone versus WBRT and radiosurgery for the treatment of brain metastases. Cochrane Database Syst Rev 2012; 9: CD006121.
Clinical presentation tends to be related to the following [1]:
- headache:
- with cognitive, memory, or behavioural symptoms; or
- with features of raised intracranial pressure:
- headache worse in the morning
- nausea/vomiting
- altered levels of consciousness, eg lethargy or somnolence in the early stages
- focal/generalised seizures
- progressive focal neurological deficits, such as:
- gradual onset weakness or sensory loss on one side of the body
- dysphasia
- unilateral visual field loss
Symptoms of a brain tumour are determined by its location and size [1].
Macmillan Cancer Support Rapid Referral Guidelines
History [1,5,6]:
- onset and duration of symptoms
- nature of headaches:
- frequency and duration
- severity
- location
- associated features, eg:
- nausea and vomiting
- worse in the morning
- relation to posture
- description of seizures if present – take a detailed history from an eye-witness if possible
- routinely ask about symptoms from likely sites for primary tumours that may spread to the brain (lung cancer, breast cancer, renal cancer, melanoma, and colorectal cancer)
- other neurological symptoms
- previous medical history
- current medications
- past history of cancer is suggestive of metastatic cancer
Examination [6]:
- perform full neurological examination − this must include assessment of visual acuity, visual fields, and fundoscopy including examining for papilloedema, the absence of which does not exclude the possibility of a brain tumour
The aim is to ensure that patients with suspected brain tumours have early access to imaging [1].
Only patients with the following symptoms can be referred on the 2WW:
- New and /or progressive neurological deficit, with or without cranial nerve palsies (please note that deafness alone cannot be defined as aneurological deficit)
- Headaches with other features of raised intracranial pressure (ICP), such as headache worse on waking, associated with vomiting, with or without papilloedema
- Previous history of cancer with unresolved headaches
GPs may wish to refer these patients directly for MRI brain, if rapid access to scanning is available, in addition to specialist referral – this would reduce the time taken to achieve a diagnosis as results would be available by the time the patient sees the specialist [6,7].
Patients with a history of cancer and any of the above symptoms should be referred back to their original treating team as a priority, as this is suggestive of metastatic disease [1].
Patients presenting with drowsiness, vomiting, or seizures may need immediate referral to A&E [7].
Consider urgent referral of patients with a rapid progression of [6]:
- subacute focal neurological deficit
- unexplained cognitive impairment, behavioural disturbance, or slowness, or combination of these
- personality changes confirmed by a witness, for which there is no reasonable explanation
- GPs may wish to refer these patients directly for MRI brain, and either wait for the MRI result before deciding on specialist referral, or make an urgent specialist referral in addition to this [7]
Consider a non-urgent referral of patients with:
- recent onset, unexplained headaches for at least 1 month but no accompanying features of raised ICP [5,6]
- MRI may be a suitable alternative to referral and GPs may wish to refer depending on these results [7]
Direct referral for MRI brain may also be of benefit in patients with the following warning symptoms [7]:
- new onset headache in patients age over 50 years, excluding patients who already have a primary diagnosis, eg migraine or temporal arteritis, unless they are resistant to treatment
- headache causing patients to wake from sleep
- new headache in patients with a history of immunocompromisation
- significant changes in headaches that have been present for some time, particularly a rapid increase in frequency
NB: Incidental abnormalities can be found on MRI in up to 10% of scans – patients should be counselled for this as it can cause considerable anxiety and have implications for life insurance or mortgage policies [3,7].
Only 1% of patients are diagnosed following an urgent GP referral for suspected cancer, with 58% of brain tumours being diagnosed via an emergency presentation [7].
Send to A&E
2WW Brain services are provided at Southmead (NBT) only
Criteria
SYMPTOMS RELATED TO THE CNS WHERE A BRAIN TUMOUR IS SUSPECTED INCLUDE:
- progressive neurological deficit
- new-onset seizures
- mental changes
- cranial nerve palsy
- previous history of cancer
- Please note that deafness alone cannot be defined as a neurological deficit
HEADACHES OF RECENT ONSET ACCOMPANIED BY FEATURES SUGGESTIVE OF RAISED INTRACRANIAL PRESSURE:
- Vomiting
- Drowsiness
- Posture related headache
- Pulse-synchronous tinnitus
- Other focal or non-focal neurological symptoms, for example blackout, change in personality or memory
- A new, qualitatively different, unexplained headache that becomes progressively severe
- Suspected recent-onset seizures (refer to neurologist)
RAPID PROGRESSION OF:
- Subacute focal neurological deficit
- Unexplained cognitive impairment, behavioural disturbances or slowness, or a combination of these
- Personality changes confirmed by a witness and for which there is no reasonable explanation even in the absence of the other symptoms and signs of a brain tumour
2WW Referral forms are available as EMIS templates
The standard RSS referral form is available as an EMIS template