Brain Tumours


This article begins with a general overview of brain tumours, including epidemiology, clinical presentation, investigations and management options. It then moves on to discuss a selection of brain tumour sub-types in some more detail.



The term brain tumour refers to an intracranial tumour affecting the brain, meninges, pituitary gland, pineal gland, cranial nerves and/or skull.



Brain tumours accounted for 3% of total cancers in 2015, but their incidence has been rising.

For adults, the highest incidence occurs in those aged between 85-89 years old.

In children less than 15 years old, brain tumours are the second most common form of cancer (acute lymphoblastic leukaemia is the most common).

Secondary brain tumours are almost three times more common than their primary counterparts.

Brain metastases can originate from cancer in many different organs:

  • Lung (most common)
  • Breast
  • Thyroid
  • Testicular
  • Renal
  • Colorectal
  • Skin (malignant melanoma)


Risk Factors

There are a variety of risk factors that can predispose an individual to develop a brain tumour.

The most widely recognised risk factors include:

  • Familial syndromes (Neurofibromatosis type 1 and 2, von Hippel-Lindau and Li-Fraumeni)
  • Ionising radiation/X-ray exposure
  • Multiple endocrine neoplasia type 1 (MEN-1)
  • Immunosuppression/EBV/HIV (primary CNS lymphoma)
  • Previous history of cancer


Overall Clinical Presentation

A brain tumour’s presentation is typically progressive and subacute in nature.

Signs and symptoms typically relate to the area of the central nervous system affected by the tumour.


Signs related to increased intracranial pressure (ICP) are common

  • Headache (worse on waking, lying down, bending forward or coughing)
  • Nausea and vomiting
  • Papilloedema


Focal neurological signs

  • Paresis of limbs (very common)
  • Speech disorders/dysphasia (very common)
  • Seizures
  • Cerebellar signs – think DASHING (dysdiadochokinesis and dysmetria, ataxia, slurred speech, hypotonia, intention tremor, nystagmus, gait abnormality)
  • Visual symptoms (bitemporal hemianopia)
  • Personality changes
  • Cranial nerve 5 palsy (common due to its long intracranial course)



  • Coma is a late stage feature of brain tumours, secondary to raised intracranial pressure.




  • A comprehensive clinical history is essential.


Physical examination

  • A comprehensive neurological assessment should be performed (including cranial nerves, upper and lower limbs)
  • Clinical examination of other systems should be performed, particularly when brain metastases are suspected (looking for the primary source).


Routine blood tests

  • Full Blood Count
  • Urea & Electrolytes
  • Calcium
  • Liver Function Tests
  • C-Reactive Protein (raised in infection and malignancy)



  • Both CT and MRI are used to help assess tumour size, adjacent structures, surrounding oedema, mass effect and secondary haemorrhage
  • A CT chest, abdomen and pelvis (CAP) should be performed if metastases are suspected



  • A biopsy is the only definitive way to diagnose a tumours tissue type (histology and cytogenetics)
  • This information can be useful in determining treatment options and prognosis

Lumbar puncture

  • Lumbar puncture should be avoided until neuroimaging has ruled out evidence of raised intracranial pressure.
  • This reduces the risk of brain herniation (commonly referred to as coning).



Management varies depending on a variety of factors, including brain tumour type, stage of disease, patient preference and current clinical condition.

Multi-disciplinary team approach

A multi-disciplinary team approach is essential, which may involve some, or all, of the specialties below:

  • Neurosurgery
  • Neurology
  • Clinical nurse specialists
  • Oncology
  • Radiology
  • Palliative care


Surgery, Radiotherapy and Chemotherapy combination therapy

  • The use of surgery, radiotherapy and chemotherapy varies depending on the type of brain tumour diagnosed.
  • Some brain tumours are particularly sensitive to chemotherapy (e.g. oligodendroglioma codeletion), whereas, for others, the only option may be complete surgical excision.
  • Often, a combination of different therapies is used to achieve the best possible outcome (e.g. adjuvant radiotherapy/chemotherapy with surgical excision).


Anticonvulsant medication

  • Anticonvulsant medication is commenced if seizure activity is present or if the tumour is in a highly epileptogenic area
  • Examples of anticonvulsant medications include Phenytoin and Levetiracetam



  • Mannitol is sometimes used to reduce intracranial pressure



  • Typically analgesia is used to reduce the severity of headaches



  • Dexamethasone is commonly used to reduce cerebral oedema around the tumour site
  • This can help reduce intracranial pressure temporarily
  • Common side effects include sleep disturbance, increased appetite, weight gain and gastro-oesophageal reflux


Ventriculo-peritoneal (VP) shunt

  • A ventriculo-peritoneal shunt may be needed if the patient presents with raised intracranial pressure secondary to hydrocephalus (a build-up of CSF in the brain)
  • This is a surgical procedure which allows diversion of CSF into the peritoneal cavity, to prevent rising intracranial pressure
  • Risks include intracerebral haemorrhage, infection and the need for further surgery if the shunt blocks



Radiation complications

  • Cognitive impairment
  • Bone growth retardation
  • Endocrine disruption
  • Hair loss
  • Nausea
  • Skin problems
  • Tissue healing problems


Neurosurgical complications

  • Paralysis
  • Seizures
  • Altered personality
  • Hydrocephalus


Chemotherapy complications

  • Nausea/vomiting
  • Anorexia
  • Alopecia
  • Pain
  • Headaches
  • Nerve damage
  • Cognitive dysfunction
  • Increased susceptibility to infections



  • The majority of benign tumours will be curable by excision.
  • Prognosis, however, is ultimately dependent on type and grade of a tumour.
  • Even with excision, GBM (glioblastoma multiforme) tumours are incurable and carry the worst prognosis of all brain tumours.



  • Usually, a slow-growing tumour that forms from the arachnoid cell cap found on the meninges
  • Incidence peak at 45
  • Usually benign and surgically viable
  • 2:1 female to male ratio
  • Includes malignant anaplastic meningioma (grade III meningioma)
  • Rarely metastasise into the body


  • Dependent on tumour size and location (can lead to mass effect symptoms)
  • Asymptomatic
  • Headache
  • Seizures
  • Spastic weakness or numbness in both legs
  • Gait abnormalities
  • Visual loss if the optic nerve is compressed


  • MRI with gadolinium contrast +/- CT
  • Angiography
CT Scan with contrast of a left-sided meningioma
Figure 1: CT Scan with contrast of a left-sided meningioma 8


  • Conservative measures unless symptomatic
  • Repeat imaging every 6-23 months
  • Surgical excision and/or radiotherapy
  • Chemotherapy has little benefit



  • 91% 5-year survival rate


Pituitary Adenoma

  • 15% of primary brain tumours
  • Occur in the pituitary gland
  • Can be functional and secrete hormones
  • Others are non-functional and cause symptoms by mass effect
  • Most are benign
  • Larger tumours (>1cm) are usually non-functional
  • Pituitary macroadenomas are the most common cause of hypopituitarism



  • Cushingoid symptoms
  • Acromegaly
  • Diabetes insipidus
  • Hyperpituitarism
  • Bitemporal hemianopia
  • Hydrocephalus
  • Amenorrhea/galactorrhea/impotence
  • Diplopia



  • Endocrine evaluation
  • MRI with gadolinium contrast +/- CT
MRI of a Pituitary adenoma
Figure 2: MRI of a pituitary adenoma 9


  • Dopamine agonists to treat prolactinomas
  • Surgery
  • Radiation


Pituitary apoplexy:

  • Sudden haemorrhage causes a rapid increase in the size of the lesion and surrounding vasogenic oedema
  • Because of mass effect, surrounding tissue necrosis occurs
  • This can result in sudden onset visual loss, headache and hydrocephalus


Acoustic Neuroma

  • Benign tumour of vestibulocochlear Schwann cells (aka vestibular schwannoma)
  • Approximately 5% of intracranial tumours
  • Presents in those over 30 years old
  • Bilateral acoustic neuromas are seen in younger patients and in those with NF type 2
  • Tumour suppressor gene abnormalities on chromosome p22 are thought to cause tumour growth


  • Gradual onset of unilateral sensorineural deafness due to compression of CN VIII
  • Headache
  • Vertigo
  • Dizziness
  • Unilateral face numbness
  • Absent corneal reflex
  • Nystagmus and gait abnormalities in larger tumours



  • Routine hearing examination useful
  • MRI with gadolinium contrast +/- CT
MRI with contrast of a right-sided acoustic neuroma
Figure 3: MRI with contrast of a right-sided acoustic neuroma (shown by arrows) 10


  • Due to the slow onset, small tumours can be monitored with annual MRI head
  • If deemed necessary, tumours can be removed surgically or with Gamma Knife radiosurgery


  • Facial palsy post-operatively
  • CSF fluid leakage after surgery – greatly reduced since the introduction of microsurgical techniques



  • 6-13% of all childhood brain tumours and 1-3% of all adult brain tumours
  • Derived from residual cells of Rathke’s pouch
  • Bimodal onset: age 5-14 and 50-70
  • Arises in the pituitary stalk
  • Can affect the hypothalamus and pituitary


  • Growth failure in children
  • Diabetes insipidus
  • Sexual dysfunction in adults
  • Hydrocephalus
  • Bitemporal hemianopia
  • Pituitary insufficiency
  • Headache


  • MRI with gadolinium contrast +/- CT
  • Endocrine testing
MRI of a Craniopharyngioma
Figure 4: MRI of a Craniopharyngioma 11


  • Surgical removal is the treatment of choice
  • Radiotherapy can be considered
  • Chemotherapy is usually of no use


Primary CNS Lymphoma

  • This is usually diffuse large B cell lymphoma
  • It accounts for <1% of all non-Hodgkin’s lymphoma sub-types



  • Seizures
  • Headache
  • Altered mental status
  • Systemic symptoms – Fever / Night sweats / Unintentional weight loss
  • Diplopia
  • Vertigo
  • Dysphagia



HIV testing:

  • CD4 count
  • Viral load



  • MRI
  • CT may be done additionally


Lumbar puncture (LP):

  • May show increased WCC /cytology positive for lymphoma



  • This may identify Epstein-Barr virus if it is present




  • Usually with Methotrexate
  • Cytarabine may be used as well
  • Chemotherapy is typically used alongside whole-brain radiation therapy



  • Typically dexamethasone



  • Tumours are typically not resectable
  • However, if mass effect is present, resection may be considered as a life-sparing procedure



  • Usually quite poor
  • In patients co-diagnosed with AIDs, median survival is 4 months with radiotherapy
  • With methotrexate-based chemotherapy, median survival is 10-18 months



  • The most common malignant tumour in children
  • Thought to arise from cerebellar stem cells between the brainstem and cerebellum near the fourth ventricle
  • Malignant primitive neuroectodermal tumour
  • Tends to seed within CSF pathways giving rise to its high metastatic propensity
  • Metastatic disease is classified by the Chang system




  • Worsening headaches and reducing level of consciousness
  • Dilated ventricles on CT head


Cerebellar signs:

  • Incoordination of limbs
  • Gait abnormalities

Mass effect symptoms:

  • Speech abnormalities
  • Vision abnormalities
  • Unilateral weakness
  • Headaches
  • Drowsiness
  • Nausea/vomiting


Extraocular muscle palsies:

  • Typically presents as diplopia



Lumbar puncture (LP):

  • This can be useful for staging
  • Only done post-operatively as it can result in brain herniation if done pre-operatively


MRI +/- CT including spinal imaging

MRI of a medulloblastoma (as indicated by arrows)
Figure 5: MRI of a medulloblastoma (as indicated by arrows) 12


  • Surgical removal of the tumour
  • Radiation
  • Chemotherapy


  • The 5-year survival for standard risk patients is around 70%



  • Gliomas account for 50% of primary brain tumours in adults
  • More common in men and in industrial countries
  • Glioma is an umbrella term for a variety of different tumour sub-types
  • They include astrocytomas, glioblastoma multiforme (GBM), pilocytic astrocytoma (PCA), ependymomas and oligodendrogliomas
  • The mean age of onset is 55 and they account for 20% of all intracranial tumours
  • PCA, on the other hand, is the commonest benign tumour in children
  • All gliomas metastasise via the cerebrospinal fluid


  • Headache
  • Vomiting
  • Seizures
  • Focal neurological deficit including visual loss
  • Cranial nerve dysfunction



  • MRI with gadolinium contrast
MRI with contrast of a GBM
Figure 6: MRI with contrast of a GBM 13



  • For all gliomas, complete excision of the tumour is often difficult
  • This is because the margins between the tumour and the brain are rarely clear
  • Biopsies taken can be used to help make a histological diagnosis
  • Carmustine implants can also be inserted; these are alkylating agents, which can help reduce the rate of tumour spread (however, they do carry a risk of serious cerebral oedema).



  • Targeted radiotherapy can be used in an attempt to shrink tumours or to destroy any residual tumour cells after excision



  • Temozolomide has been shown to increase survival when given alongside surgery and/or radiotherapy



  • Overall, the prognosis is poor as gliomas are rarely curable
  • As an example, GBMs have a mean life expectancy of 1 year
  • PCAs are an exception, with more than  95 % of patients surviving 5 years


Edited by

Samantha Strickland

Hull York Medical School


Reviewed by

Konstantinos Lilimpakis

Neurosurgical Clinical Fellow


  1. DN Louis, A Perry, G Reifenberger, A von Deimling, D Figarella-Branger, W KCavenee et al (2016) ‘The 2016 World Health Organization Classification of Tumours of the Central Nervous System: a summary’, Acta Neuropathologica, 131(6), pp. 803-820.
  2. Whittle IR, Smith C, Navoo P, Collie D (2004), ‘Meningiomas’, Lancet, 363, pp. 1535-1543.
  3. BMJ Best Practice (2017) ‘Overview of brain tumours’, BMJ Best Practice. Available at:
  4. Cancer Research UK (2018) ‘Brain, other CNS and intracranial tumours incidence statistics’, Cancer Research UK. Available at:
  5. National Institute for Health and Care Excellence (2006) ‘Improving outcomes for people with brain and other central nervous system tumours’, NICE guideline (CSG10)
  6. Grant R (2004) ‘Overview: brain tumour diagnosis and management/RoyalCollege of Physicians guidelines’, J Neurol Neurosurg Psychiatry 75(2), pp. 18-23
  7. Hamilton W, Kernick D (2007) ‘Clinical features of primary brain tumours: a case-control study using electronic primary care records’, Br J Gen Pract 57(542), pp. 695-699
  8. Taken from:
  9. Taken from
  10. MRT-Bild. 2009. Taken from:
  11. Taken from:
  12. The Armed Forces Institute of Pathology. Taken from:
  13. Christaras A.


Print Friendly, PDF & Email