Brain Tumours

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Brain tumours\: intracranial tumours a
Incidence\: 3% of total cancers in 2015, highest in adults aged 85-89 years, second most common cancer in children under
15.
Secondary brain tumours\: three times more common than primary brain tumours.
Common symptoms of brain tumours\: headache (worse on waking, lying down, bending forward, coughing), nausea,
vomiting, papilloedema, and coma.
Meningioma\: slow-growing tumour from arachnoid cells, peak incidence at 45 years, 2\:1 female to male ratio, usually
benign.
Symptoms\: headache, seizures, spastic weakness or numbness in legs, gait abnormalities, visual loss if optic nerve
compressed.
Investigations\: MRI with gadolinium contrast, CT, angiography.
Management\: usually conservative, surgical excision, radiotherapy.
Prognosis\: good, 91%
Pituitary adenoma\: occurs in pituitary gland, 15% of primary brain tumours, can be functional (hormone-secreting) or non-
functional (mass e
Symptoms\: cushingoid symptoms, acromegaly, diabetes insipidus, hyperpituitarism,
amenorrhea/galactorrhoea/impotence, bitemporal hemianopia, hydrocephalus, diplopia.
Investigations\: endocrine tests, MRI with gadolinium contrast, CT.
Management\: dopamine agonists (for prolactinomas), surgery, radiation therapy.
Complication\: pituitary apoplexy (haemorrhage, rapid increase in tumour size, necrosis).
Acoustic neuroma (vestibular schwannoma)\: benign tumour of vestibulocochlear Schwann cells, 5% of intracranial
tumours, usually in people over 30, bilateral in neuro
Symptoms\: unilateral sensorineural hearing loss, tinnitus, dizziness, disequilibrium, facial pain/numbness, facial weakness,
headaches, nausea, vomiting.
Investigations\: audiometry, MRI with gadolinium contrast, CT.
Management\: conservative for small tumours, surgical removal, stereotactic radiosurgery.
Complications\: post-operative facial palsy, CSF leakage after surgery.
Craniopharyngioma\: arises in the pituitary stalk, a
Symptoms\: growth failure (children), diabetes insipidus, sexual dysfunction (adults), hydrocephalus, bitemporal hemianopia,
pituitary insu
Investigations\: MRI with gadolinium contrast, CT, endocrine tests.
Management\: surgical removal, radiotherapy.
Primary CNS lymphoma\: usually di
Symptoms\: seizures, headache, altered mental status, systemic symptoms (fever, night sweats, weight loss), diplopia,
vertigo, dysphagia.
Investigations\: MRI, CT, HIV testing, lumbar puncture, Epstein-Barr virus PCR.
Management\: chemotherapy (methotrexate, cytarabine), radiation therapy, steroids (dexamethasone), surgery (if mass
e
Prognosis\: poor, median survival 4 months with radiotherapy, 10-18 months with methotrexate-based chemotherapy.
Medulloblastoma\: most common malignant tumour in children, arises from cerebellar stem cells, seeds within CSF
pathways.Symptoms\: hydrocephalus, cerebellar signs, mass e
Investigations\: lumbar puncture, MRI, CT including spinal imaging.
Management\: surgical removal, radiotherapy, chemotherapy.
Prognosis\: 70%
Glioma\: includes astrocytomas, glioblastoma multiforme (GBM), pilocytic astrocytoma (PCA), ependymomas,
oligodendrogliomas.
Symptoms\: headache, vomiting, seizures, focal neurological de
Investigations\: MRI with gadolinium contrast.
Management\: surgery (biopsies, carmustine implants), radiotherapy, chemotherapy (temozolomide).
Prognosis\: poor, mean life expectancy one year for GBMs, >95%
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A comprehensive topic overview

Introduction

The term brain tumour refers to an intracranial tumour a
nerves and/or skull.
In 2015, brain tumours accounted for three percent of total cancers, however, their incidence has been rising.
In 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 cancer (acute lymphoblastic leukaemia is
the most common).
Secondary brain tumours are three times more common than primary brain tumours.
Clinical features of brain tumours
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 a
Signs related to raised intracranial pressure (ICP) are common, these include\:
Headache (worse on waking, lying down, bending forward or coughing)
Nausea and vomiting
Papilloedema
Coma\: a late-stage feature of brain tumours, secondary to raised intracranial pressure

Meningioma

A meningioma is a slow-growing tumour that forms from the arachnoid cell cap found on the meninges.
The peak incidence occurs at 45 years of age, and there is a 2\:1 female to male ratio.
Meningiomas are usually benign and surgically viable. They rarely metastasise into the body.
Clinical features
The clinical presentation is dependent on tumour size and location (can lead to mass e
Many patients are asymptomatic with the meningioma detected on brain imaging performed for another indication.
Clinical features may include\:Headache
Seizures
Spastic weakness or numbness in both legs
Gait abnormalities
Visual loss if the optic nerve is compressed
Investigations
Relevant investigations include\:
MRI with gadolinium contrast +/- CT
Angiography
Figure 1. A CT scan with contrast of a left-sided meningioma.
8
Management
Management is usually conservative unless symptoms develop. Follow-up imaging is used to monitor the meningioma.
If intervention is required, options include surgical excision and/or radiotherapy. Chemotherapy has little bene
Prognosis
Meningiomas have a good prognosis with a 91%

Pituitary adenoma

Pituitary adenomas occur in the pituitary gland and are responsible for 15% of primary brain tumours.
They can be functional and secrete hormones. Larger tumours (>1cm) are usually non-functional and cause symptoms by
mass e
Pituitary macroadenomas are the most common cause of hypopituitarism.
Clinical features
Functional pituitary adenomas can cause a variety of clinical presentations including\:
Cushingoid symptoms
Acromegaly
Diabetes insipidus
HyperpituitarismAmenorrhea/galactorrhoea/impotence
Clinical features related to the mass e
Bitemporal hemianopia\: due to compression of the optic chiasm
Hydrocephalus
Diplopia
Investigations
Relevant investigations include\:
Endocrine laboratory tests
MRI with gadolinium contrast +/- CT
Figure 2. MRI of a pituitary adenoma.
9
Management
Management of pituitary tumours may include\:
Dopamine agonists to treat prolactinomas
Surgery
Radiation therapy
Complications
Pituitary apoplexy is a serious complication occurring when a haemorrhage causes a rapid increase in the size of the
lesion and surrounding vasogenic oedema.
Due to the mass e
hydrocephalus.

Acoustic neuroma

An acoustic neuroma is a benign tumour of vestibulocochlear Schwann cells (a.k.a. vestibular schwannoma).
Acoustic neuromas account for approximately 5% of intracranial tumours and usually present in people over 30 years old.
Bilateral acoustic neuromas are seen in younger patients and in those with neuro
Clinical featuresTypical symptoms of acoustic neuroma include\:Unilateral sensorineural hearing loss (SNHL) and/or tinnitus\: 90% of patients present with progressive SNHL, whereas
only 5% present with sudden SNHL
Dizziness and disequilibrium (vestibular dysfunction)
Other symptoms can include\:
Facial pain and numbness\: trigeminal (CN V) nerve compression
Facial weakness\: facial (CN VII) nerve compression
Headaches, nausea and vomiting\: suggestive of raised intracranial pressure
Other important areas to cover in the history include\:
Past medical history or family history of similar tumours (if considering NF2)
Investigations
Relevant investigations include\:
Audiometry\: to con
MRI with gadolinium contrast +/- CT
Figure 3. MRI with contrast of a right-sided acoustic neuroma (shown by arrows)
10
Management
Management of small tumours may be conservative with annual follow up imaging.
If intervention is required, tumours can be removed surgically or with stereotactic radiosurgery.
Complications
Complications include\:
Post-operative facial palsy
CSF
For more information, see the Geeky Medics guide to acoustic neuroma.

Craniopharyngioma

Craniopharyngiomas arise in the pituitary stalk and can a
residual cells of Rathke’s pouch.They are responsible for 6-13% of all childhood brain tumours and 1-3% of all adult brain tumours. There is a bimodal onset
at age 5-14 and 50-70.
Clinical features
Clinical features may include\:
Growth failure in children
Diabetes insipidus
Sexual dysfunction in adults
Hydrocephalus
Bitemporal hemianopia
Pituitary insu
Headache
Investigations
Relevant investigations include\:
MRI with gadolinium contrast +/- CT
Endocrine laboratory investigations
Figure 4. MRI of a Craniopharyngioma
11
Management
Management options for craniopharyngioma include\:
Surgical removal\: the treatment of choice
Radiotherapy can be considered
Chemotherapy is usually of no bene

Primary CNS lymphoma

Primary CNS lymphoma is usually di
subtypes.
Clinical featuresClinical features include\:Seizures
Headache
Altered mental status
Systemic symptoms\: fever, night sweats, unintentional weight loss
Diplopia
Vertigo
Dysphagia
Investigations
Relevant investigations include\:
MRI +/- CT
HIV testing\: CD4 count, viral load
Lumbar puncture\: may show increased WCC and/or cytology positive for lymphoma
Epstein-Barr virus PCR\: may identify Epstein-Barr virus if it is present
Management
Management options for primary CNS lymphoma include\:
Chemotherapy\: usually methotrexate, cytarabine may be used in addition
Radiation therapy\: whole-brain radiation therapy alongside chemotherapy
Steroids\: dexamethasone
Surgery\: tumours are typically not resectable. However, if mass e
sparing procedure.
Prognosis
The prognosis is usually poor. In patients co-diagnosed with AIDS, median survival is four months with radiotherapy. With
methotrexate-based chemotherapy, median survival is 10-18 months.

Medulloblastoma

Medulloblastoma is the most common malignant tumour in children. It is thought to arise from cerebellar stem cells
between the brainstem and cerebellum near the fourth ventricle.
Medulloblastoma tends to seed within CSF pathways giving rise to its high metastatic propensity. Metastatic disease is
classi
Clinical features
Clinical features include\:
Hydrocephalus\: worsening headaches and decreased level of consciousness
Cerebellar signs\: incoordination of limbs, gait abnormalities
Mass e
nausea/vomiting
Extraocular muscle palsies\: typically presents as diplopia
Investigations
Relevant investigations include\:
Lumbar puncture (LP)\: performed post-operatively and useful for staging
MRI +/- CT including spinal imagingFigure 5. MRI of a medulloblastoma (as indicated by arrows).
12
Management
Management options for medulloblastoma include\:
Surgical removal of the tumour
Radiotherapy
Chemotherapy
Prognosis
The

Glioma

Glioma is an umbrella term for a variety of di
multiforme (GBM), pilocytic astrocytoma (PCA), ependymomas and oligodendrogliomas.
Gliomas account for 50% of primary brain tumours in adults and they are more common in men and in industrial countries.
The exception is PCA, which is the commonest benign tumour in children.
The mean age of onset is 55 and they account for 20% of all intracranial tumours. All gliomas metastasise via the
cerebrospinal
Clinical features
Clinical features include\:
Headache
Vomiting
Seizures
Focal neurological de
Cranial nerve dysfunction
Investigations
Relevant investigations include\:
MRI with gadolinium contrastFigure 6. MRI with contrast of a GBM
13
Management
Surgery
For all gliomas, complete excision of the tumour is often challenging. 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).
Radiotherapy
Targeted radiotherapy can be used to shrink tumours or to destroy any residual tumour cells after excision.
Chemotherapy
Temozolomide has been shown to increase survival when given alongside surgery and/or radiotherapy.
Prognosis
year.
Overall, the prognosis is poor as gliomas are rarely curable. As an example, GBMs have a mean life expectancy of one
PCAs are an exception, with more than 95% of patients surviving

References

DN Louis, A Perry, G Reifenberger, A von Deimling, D Figarella-Branger, W KCavenee et al (2016) 'The 2016 World Health
Organization Classi
, Acta Neuropathologica, 131(6), pp. 803-
820.
Whittle IR, Smith C, Navoo P, Collie D (2004), Meningiomas, Lancet, 363, pp. 1535-1543.
BMJ Best Practice (2017) Overview of brain tumours BMJ Best Practice. Available from\: [LINK]
Cancer Research UK (2018) Brain, other CNS and intracranial tumours incidence statistics’
, Cancer Research UK. Available
from\: [LINK]
National Institute for Health and Care Excellence (2006) ‘Improving outcomes for people with brain and other central
nervous system tumours’
, NICE guideline (CSG10)
Grant R (2004) ‘Overview\: brain tumour diagnosis and management/Royal College of Physicians guidelines’
, J Neurol
Neurosurg Psychiatry 75(2), pp. 18-23Hamilton 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
Glitzy queen00. Contrast enhanced meningioma. License\: [Public domain]
HellerhoCC-BY]
MRT-Bild. A k u s t i k u s n e u r i n o m M r t . License\: [Public domain]
HellerhoCC-BY]
The Armed Forces Institute of Pathology. Medulloblastoma. License\: [Public domain]
Christaras A. G l i o b l a s t o m a . License\: [CC-BY]

Reviewer

Konstantinos Lilimpakis
Neurosurgical Clinical Fellow

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Contents

Introduction
Meningioma
Pituitary adenoma
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