Current perspectives on

childhood brain tumours: a

review
DS Abubakar
HC Traunecker
Abstract
Tumours of the brain and spinal cord are the commonest solid tumour in
children and the leading cause of cancer related morbidity and mortality.
The increasing availability of modern imaging techniques has resulted in
peak of incidences in the 1990s. Current challenges for the general public,
paediatricians and general practitioners lie in increasing awareness of the
presenting features so that an earlier diagnosis can be made. Specic is-
sues and peculiarities relating to congenital/infantile brain tumours have
been explored. Improvedhistological classication supportedby biological
markers and subgroup allocation has enabled better treatment planning
and increased survival. The emphasis is now focussing on formulating
treatment strategies associated with reduced risks of toxicities for favour-
able risk groups and intensication of treatment for those with worst prog-
nosis. The interface between brain tumours and epilepsy, effects of anti-
epileptic medications, antineoplastic medications and tumour surgery on
epilepsy were touched upon. Advances in radiotherapy through the
increasing availability of proton beam radiotherapy and its advantage
over photon radiation therapy in brain tumours has also been discussed.
Keywords anti-epileptic drugs; antineoplastics; brain tumour; brain
tumour surgery; chemotherapy; children; congenital; epilepsy surgery;
infantile; magnetic resonance imaging; photon beam; proton beam;
radiotherapy; tumour biology; ultrasonography
Introduction
Brain and spinal tumours are the most common solid tumours in
children. They are also the leading cause of childhood cancer
related mortality. Although most cases arise sporadically, how-
ever, a small percentage is known to be associated with cancer
predisposition syndromes like neurobromatosis type 1 or 2
(Table 1). The increasing availability of magnetic resonance im-
aging (MRI) facilities has lead to improved diagnosis, ascertain-
ment of metastases and planning of treatment. This has eventually
led to a peak in incidence in the 1990s. Clinical treatment strategies
have also evolved in the last decade, reecting ner reclassica-
tion and new denition of risk groups, based on histologic subsets
and the use of biological and molecular techniques.
The combination of the above, coupled with advancement of
surgical techniques and adjuvant chemotherapy has lead to
improved 5-year survival of childhood brain tumours e for
example exceeding 75% and 96 % in medulloblastomas and low
grade gliomas respectively. This has lead to an emphasis on
reducing long-term morbidity in favourable risk groups by
exposure to less toxic chemotherapy and radiation therapy. This
is in contrast to poor outcome in less favourable subsets for
example malignant gliomas with less than 20% ve year survival
despite intensication of chemotherapy and radiation therapies.
Emphasis on early detection through early recognition of
symptoms and signs by both public and healthcare professionals is
a recent strategy to reduce the late effects burden and improve
outcomes. Improved long term survival after treatment is opening
up further challenges to paediatricians and physicians who have to
manage the health needs of an ever increasing population of sur-
vivors of brain tumours whose ageing process is accelerated due to
the effects of surgery, chemotherapy and or radiotherapy.
Epidemiology
Incidence has remained stable worldwide ranging between 29.9
and 47.1 per million in children aged 0e15 years. This variation
between regions (Asia, America and Europe) is reective of
limitations in denition of histologic types and whether di-
agnoses are based only on histology or combination of histology
and imaging criteria.
Environmental factors
Apart from ionising radiation, there is a lack of certainty as to
environmental inuences causing a brain tumour in children.
Marked increased risks of brain tumour have been linked to
exposure to ionising radiation as exemplied by a 30-fold
increased incidence of head and neck tumours and 9-fold
increased risk of meningiomas following low dose exposure to
ionising radiation for the treatment of benign conditions such as
e tinea capitis. Higher dose radiation therapy for childhood
leukaemia has been associated with a 21.7-fold increased risk of
brain tumours over a period of 16 years in a cohort of 9720
children, of which a majority were malignant.
However, other environmental exposures are for example low
frequency microwave irradiation, viral infections (JC virus or
SV40) or cured meats ingestion have not been proven with any
degree of certainty. Although there are no published studies
about risks of mobile phones and brain tumours in children, the
evidence in adults does not support any increased risk, at least
on short term use of mobile phones.
Clinical presentation
Presentation varies with age, tumour type, location and also with
the rate of growth. Practitioners should be aware of intermittent
symptoms/signs and also of non-localising features in brain tu-
mours in infants and those aged less than 3 years. Most often, they
are typied by loss of developmental milestone, failure to thrive,
macrocephaly with intermittent sun-setting and irritability.
Localising symptoms and signs are typically seen in children
older than 3 years as detailed in Table 2. In general, benign tu-
mours (e.g. cerebral and cerebellar astrocytomas) tend to grow
slowly, giving rise to a lag time between onset of symptoms and
nal diagnosis, whereas malignant tumours (e.g. malignant
DS Abubakar MBBS DCH MRCPCH Specialty Registrar, University Hospital of
Wales, Heath Park, Cardiff, UK. Conicts of interest: none.
HC Traunecker MRCP PHD Consultant Paediatric Oncologist, University
Hospital of Wales, Heath Park, Cardiff, UK. Conicts of interest: none.
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supratentorial gliomas, diffuse brain-stem gliomas and medul-
loblastomas) grow rapidly with manifestation of symptom-
atology within days or weeks.
HeadSmart be brain tumour aware
Following concerns by healthcare professionals and parents
regarding the delay between onset of symptoms and diagnosis of
a brain tumour in children within the UK, a collaborative
campaign was initiated to raise awareness. This was started in
June 2011 between the Royal College of Paediatrics and Child
Health, the Cancer Charity Samantha Dickson Brain Tumour
Trust now called the Brain Tumour Charity and the brain tumour
research centre based at University of Nottingham (http://www.
cbtrc.org). The aim is to reduce the delay in diagnosis from 3
months to 1 month, making it comparable to other European
countries. An initial report had indicated an increased awareness
of symptomatology by paediatrician within 4 months of the onset
of the campaign.
The programme involves identifying clinical and community
champions to promote public and professional awareness, using
monitoring of symptom interval as a driver for change and to
disseminate information that will raise the prole of brain
tumour awareness. Twenty clinical champions were identied
within the UK and the Republic of Ireland. A multipronged
approach has been established using social media, media cam-
paigns, symptom cards (similar to meningococcal disease) and
liaison with professional bodies e Royal Colleges of Paediatrics
and General Practice with changes in health policy. It has been
shown to raise awareness, particularly for low grade tumours
where prolonged delays could lead to more serious physical
disability and visual symptoms. It is hoped that earlier diagnosis
and initiation of treatment will minimise or even prevent their
development. This campaign has generated 14 million television
viewing (11% of UK population), 95,500 website hits (more than
6000 per month in 2013), distribution of more than half a million
symptom cards, 25,000 likes on Facebook and 1,200 strong
followership on Twitter. There is 70% increased awareness by
surveyed paediatricians who reported a 22% increased con-
dence in identifying symptoms and signs of a brain tumour. In
addition, this has also lead to the National Institute of Health and
Clinical Excellence (NICE) developing referral guidelines and the
National Cancer Intelligence Network (NCIN) to start collecting
data on the time between onset of symptoms/signs to diagnosis
(total diagnostic interval e TDI).
A recent data analysis e in May 2013 has shown an encour-
aging improvement due to the HeadSmart campaign and its
preparatory work. The median time to intervention in 2006 (rst
data collections), 2011 (pre launch) and 2013 (post launch)
dropped from 14.4 to 9.1 and recently reached 6.9 months with
the mean time suggesting a reduction in outliers as well (35.3
e24.2 and 20.4 months). This is a signicant reduction in time to
diagnosis for all 3 time points (Personal communication from
Prof. David Walker).
Future attention of HeadSmart will be directed to building
awareness within primary care and the public, utilising commu-
nity champions, revisiting the guidelines and ensure mandatory,
population based data collection via NCINto determine the impact
of reduced TDI onpatient outcomes and to disseminate HeadSmart
principles internationally. For detailed information regarding
HeadSmart, kindly refer to http://www.headsmart.org.uk.
Tumour location and common presenting signs and symptoms
Cerebral hemisphere Diencephalic Posterior fossa Brain stem Pineal region
Hemiparesis
Seizures
Hemisensory decits
Visual eld defects
Headaches
Visual loss/eld defects
Endocrinopathy
Behavioural changes
Headaches
Ataxia/dysmetria
Headaches
Nausea/vomiting
Neck pain
Extra ocular palsies
Extra ocular palsy
Facial palsies
Swallowing difculty
Hemi/quadriparesis
Ataxia/dysmetria
Ataxia
Extra ocular palsy
Headaches
Table 2
Genetic syndromes and associated brain tumours
Syndrome Gene affected Cancer association
Gorlin PTCH1 Meduloblastomas
LieFraumeni TP53 Many malignant brain tumours
Neurobromatosis 1 NF1 Gliomas
Neurobromatosis 2 NF2 Meningiomas, Neurilemomas, Ependymomas
Tuberous sclerosis TSC1, TSC2 Subependymal giant cell astrocytomas
Turcot APC, various mismatch repair genes Many malignant brain tumours
Von HippeleLindau VHL Haemangioblastomas
Table 1
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Difculties in diagnosing congenital/infantile brain tumours
Certain characteristics of intracranial tumours in infancy are due
to unfused cranial sutures, allowing unrestricted tumour growth
and displacement of normal brain tissue and in addition to its
rarity will lead to difculties in diagnosis.
The incidence of congenital/infantile brain tumours is very
low, ranging from 0.5% to 4% of all paediatric brain tumours,
however, is associated with a high fatality rate accounting for up
to 20% of all deaths resulting from neoplasm during this period
of life. Unlike brain tumours in older children and adolescents,
congenital brain tumours are mainly supratentorial as opposed to
infratentorial in the older child. There is also a predominance of
non-malignant teratoma which is very rare in the older age
group. Congenital brain tumours also differ in their histologic
type and clinical course/prognosis when compared to brain tu-
mours in older children and adults.
Antenatal detection is commonly an incidental nding during
ultrasonography (USS) for other reasons; hence only about 18%
of infantile brain tumours are picked up in-utero. Magnetic
resonance imaging (MRI) scans can help in improving antenatal
detection and may inform treatment decisions relating to the
birth. Intracranial tumours are usually depicted as calcied re-
gions on USS or as a heterogenous mass with associated hydro-
cephalus on MRI (Figure 1).
Presentations after birth are due to raised intracranial pressure
(bulging fontanelle, sun setting phenomenon, irritability, apnoea,
failure to thrive, drowsiness and vomiting), neurologic decits and
on rare occasion, may present with seizures or intraventricular
haemorrhage. Commonly, patients will be asymptomatic but
present with excessive head circumference; beyond 99
th
centile or
disproportionate head circumference when compared with weight
and length for age or a head circumference that is rapidly crossing
centiles in the rst few months of life.
Histologically, the common types are: teratoma (up to 36.5%)
astrocytoma (28.9%) primitive neuroectodermal tumour (PNET)
(26.7%), choroid plexus papilloma (15.4%) and glioblastoma
multiforme (14.6%). Certain histologic types like medulloblastoma
behave more aggressively ininfancy thantheir typical characteristic
in older children, and contrastingly, high grade astrocytoma be-
haves more benignly in infancy. In infancy, higher risks are asso-
ciated with all treatment options compared to older children. Life
threatening haemorrhages during curative surgery and increased
toxicity during adjuvant chemotherapy are common. An unac-
ceptable side effect spectrumis associated with radiotherapy which
limits treatment options compared to older children.
The overall prognosis for congenital brain tumours is bleak
with postnatal survival of only 28%. In one large series, one third
of babies were still born and another third only survived to the
rst week of life. Neonates with a choroid plexus papilloma,
ganglioglioma or low grade astrocytoma have a better prognosis
as opposed to a teratoma or a primitive neuroectodermal tumour.
Signicant cognitive decits have also been demonstrated in
long term survivors of childhood brain tumours. Risk factors
associated with increased morbidity include; younger age at
diagnosis, tumour site at cerebral hemisphere, hydrocephalus
requiring treatment with a shunt and radiation therapy.
Epilepsy in paediatric brain tumours
Seizures may be encountered as presenting features, at diagnosis,
during treatment, at progression and during the palliative phase
of a brain tumour. Seizures are a common complication of
treatment for brain tumours in up to 15% of cases. On the other
hand, brain tumours are only found in 1e3% of new onset sei-
zures. Seizures as the presenting symptom occur only in the
minority of brain tumours as most brain tumours in children are
infra-tentorial. The incidence is related to tumour histology and
supratentorial location. Whereas slow growing tumours like
dysembryoplastic neuroepithelial tumour and some low grade
astrocytoma have the highest risk of seizures (75e100%), faster
growing ones like meningioma and very fast growing high grade
astrocytoma, have a 30e60% incidence of seizures although they
are less common in children compared with adults.
Seizures as the single presenting feature of a brain tumour are
associated with a favourable prognosis unlike when associated
with signs of raised intracranial pressure or neurologic decits.
Seizure escalation after a period of stabilisation may indicate
malignant transformation of a low grade tumour to a high grade
tumour, again an observation more commonly observed in adults.
Evaluation of seizures in children with known brain tumour
Although most oncologists are initially concerned about tumour
relapse or progression when new seizures emerge in the context
of a patient with a known brain tumour, other conditions warrant
ruling out (Table 3). These include electrolyte abnormalities,
infections, and disorders of sodium homoeostasis, neurotoxicity
from radiation therapy or chemotherapy. Other considerations
include cerebrovascular events due to hypercoagulability and
radiation damage to vessels, low anti-epileptic drug levels sec-
ondary to drugedrug interactions or poor compliance. Seizures
may be an entirely new phenomenon unrelated to the existing
tumour, and full characterisation including EEG will be required.
Late onset seizures
Seizures occurring 5 years after diagnosis of a brain tumour have
been studied retrospectively in a large collaborative cohort. Up to Figure 1 Choroid plexus carcinoma with associated severe hydrocephalus
in an infant. Reprinted from Hani Alsalam with kind permission.
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25% of these long term survivors have seizures as a sequel, and
only 6.5% of these will have new onset seizures at least 5 years
after diagnosis. There is a similar incidence for both PNET and
astroglial tumours for late onset seizures. Radiation therapy to
the cerebral cortex using a dose in excess of 30 Gy is associated
with an up to 2-fold increased risk. Increased risks of seizures
were also associated with a younger age of the child at the time of
radiotherapy.
Seizure management
Considerations for anti-epileptic drug (AEDs) therapy in children
with a brain tumour are summarised in Table 4. It is worth
noting the tendency to treat the rst unprovoked seizure espe-
cially if there is associated epileptiform abnormality in children
with a brain tumour. There is a preference for non-enzyme-
inducing AEDs like gabapentin, levetiracetam and lacosamide as
opposed to carbamazepine, phenytoin and phenobarbitone.
Antineoplastic effects of AEDs
It has recently been shown that valproate has antineoplastic ac-
tion via its effect as anti-histone deacetylase inhibitor leading to
induction of cell cycle arrest, differentiation and apoptosis
in vitro. Recent studies have also demonstrated anti-tumour ef-
fect of valproate in combination with other chemotherapy and
radiation therapies. The ndings of enhanced survival when
valproate and an oral chemotherapeutic medication, temozolo-
mide, were used together with radiotherapy for glioblastoma has
lead to research interest in valproate as adjuvant therapy in brain
tumours in adults and children.
Antiseizure effects of antineoplastics
Independent of their effect on tumour size, it has recently been
shown that some antineoplastic agents i.e. e temozolomide used
together with AED lead to better seizure control. This is thought
to be mediated via biological changes in the tumour and sur-
rounding epileptogenic focus, however, further research in this
eld is warranted. The anticipated limitations of such therapy are
likely to be due to the short term and long term side effects of
antineoplastic agents.
Brain tumours and epilepsy surgery
Intractable seizures are well recognised in children with a brain
tumour. A recent study has demonstrated improved seizure
outcome with 75% of cases being seizure free at 1e2 years post
resection of a low grade glioma. Factors predicting seizure
freedom after surgery are as follows: gross total resection, good
preoperative seizure control and duration of seizures of 1 year or
less, whereas simple partial seizures were associated with the
worst rate of postoperative seizure control. Further dedicated,
specialised epilepsy surgery may be needed for patients with
persistent seizures despite medication and tumour resection.
Recent advances in paediatric brain tumours
Advances in understanding tumour biology
Over the last 5e10 years, the biggest advances in paediatric brain
tumours have been made in dening tumour specic biological
markers and their potential prognostic signicance. This is an
excellent example of translational research, taking place on a
cooperative and international basis. The overall aim is to offer
treatment schedules with less neurotoxic therapy to patients in a
favourable prognosis group and intensication of treatment for
those with poorer prognoses, accepting higher levels of long term
sequelae. By doing so, the late deleterious effects on cognition,
living skills and incidences of secondary cancers could be
reduced in the patient groups with prognostic markers suggestive
of higher cure rate.
Signicant improvement in the understanding of biological
denition of medulloblastoma has been achieved while the
clinical management of medulloblastoma has improved in the
past decade. The average survival of 60% in the 80s/90s was
greatly advanced to 80e85%. This was achieved mainly by
focussing on better surgical resection, improved clinical risk
stratication, routine use of chemotherapy and better radio-
therapy planning. Four biologically distinct groups of medullo-
blastoma have been dened with sufcient certainty for research
into treatment strategies for medulloblastoma focussing on up-
front biological risk stratication and therapy adaptation in
large, international cohorts of patients.
Furthermore, reclassication of previously difcult to dene
histological types of tumours has occurred based on cytogenetic
information. Rhabdoid/atypical teratoid tumours that account
for 10e15% of infant embryonal tumours with previously
Causes of seizures in children with brain tumours -
adapted from Wells EM et al., with kind permission
Tumour relapse
Tumour progression
Secondary malignant
neoplasm
Posterior fossa syndrome
Toxic encephalopathy
Sub therapeutic AED levels
Hyponatraemia (SIADH, CSW)
Hypernatraemia (DI)
Hypoglycaemia
Infections (CNS/shunt)
Cerebrovascular events
New epilepsy unrelated
to tumour
AED, anti-epileptic drug; CNS, central nervous system; CSW, cerebral salt
wasting, DI, diabetes insipidus.
Table 3
Considerations for AEDs in children with brain tumours;
adapted from Wells EM. et al., with kind permission
No proven benet of AED prophylaxis
Up to 20e40% incidence of AED side effects in patients with brain
tumours
For patients with perioperative seizures, convention is to stop AEDs
3 months after gross total resection if no postoperative seizures
and uncomplicated course
Enzyme-inducing AEDs (e.g., carbamazepine, phenytoin, and
phenobarbital) increase drugedrug interactions and may be pro-
hibited by research protocols
Valproic acid may have antitumoral effects, but thrombocytopenia
and platelet dysfunction may occur
AED discontinuation may be attempted after 2e3 years of seizure
freedom, but recurrence is higher than in epilepsy without structural
lesion
Table 4
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abysmal prognosis, have beneted from improved diagnostic
certainty based on cytogenetic markers and tumour specic
strategies resulting in improved outcome.
The pathological differential diagnosis of low grade astrocy-
toma can be difcult to ascertain in childhood. The recently
dened BRAF oncogene allows differentiation between intrinsi-
cally aggressive variants versus the more common indolent, non-
aggressive tumours. Especially when deciding on therapy for
tumours in sites that are not amenable to surgery, the biological
marker will help stratify therapy according to risk.
Proton beam therapy
Conventional radiotherapy usually delivers high doses of ionis-
ing radiation to the tumour target but also low to intermediate
doses to surrounding normal tissue in the exit area of the radi-
ation beam, hence has the inherent danger of adverse, long term
radiation side effects to normal tissues. These long term side
effects include: delayed physical, neurocognitive, musculoskel-
etal and pubertal development. More specically, hearing loss,
effects on hypothalamoepituitary axis and causation of second
malignancy are well recognised. Proton beam radiation therapy
seeks to minimise the above dangers by delivering high charged
particle radiation doses to target tissues while minimising expo-
sure to surrounding normal tissues (Figure 2).
Critically sensitive sites within the brain are for example the
brain stem, optic structures, non-diseased brain tissues, hearing
apparatus, pituitary glands and hypothalamus may thus be
spared the intolerable radiation doses that are necessary to treat
most intracranial tumours. Similarly, in treating spinal cord tu-
mours, normal tissues within chest and abdomen are protected
from unnecessary exposures to high doses radiation. Improved
cognitive effects and mitigation of growth hormone deciency
have been predicted on modelling research designed to compare
proton therapy to conventional radiotherapy for children with
medulloblastoma.
In the treatment of low grade glioma that required surgical
excision plus immediate or delayed radiation therapy, a clear
survival advantage of a symptom free period was shown in the
Figure 2 Comparing proton to photons radiation beam, reprinted from Nicholas S Boehling et al., with kind permission. Axial, coronal and sagittal isodose
distributions comparing intensity-modulated proton therapy (IMPT) (left), three dimensional conformal proton therapy (3D-PRT) (middle) and intensity-
modulated radiotherapy (IMRT) (right).
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latter compared to the former group. Proton therapy is especially
benecial to paediatric patients with pilocytic astrocytoma if
other treatment options like surgical excision or chemotherapy
are not suitable. This paediatric tumour is associated with a long
life expectancy and therefore, treatment modalities such as pro-
ton therapy is particularly important in minimising long term
toxicities that are associated with morbidity and increased
mortality.
A recent study of early outcome after adjuvant proton therapy
in a small cohort of 15 children with medulloblastoma and
supratentorial PNET has shown excellent outcome of disease
control after the short follow up at a median of 31 months post
irradiation. Moreover, reduced toxicities in the form of ototoxicy
in 2 out of 15 cases and endocrinopathy in 2 out of 15 cases have
been observed.
There are also indications that proton radiation modality may
lead to a lower risk of secondary cancers compared to conven-
tional (photon) radiation using modelling analysis. In a recent
study a risk ratio reduction of 14%, 18%, 12%, 23% and 31%
was observed for patients suffering from a brain tumour, medi-
astinal Hodgkin lymphoma, neuroblastoma, pelvic rhabdomyo-
sarcoma and medulloblastoma respectively when proton beams
were used as opposed to conventional radiation therapy using
photons.
Facilities for proton beam therapy do not currently exist in the
UK although 2 sites, in London and Manchester, have been
identied as future centres to deliver this modality of treatment
by 2017. Since 2008, there has been a steady increase in the total
number of adult and paediatric patients going abroad for such
treatment peaking at 79 in 2011/12. A Proton Clinical Reference
Panel has been set up to manage referral pathways in England,
advising the funding bodies in Scotland, Wales and Northern
Ireland on the indication of such treatments.
Using strict criteria, the following paediatric brain tumour
diagnoses are considered for proton beam therapy abroad; skull
base chordoma and chondrosarcoma, ependymoma, optic
pathway glioma, craniopharyngioma and pineoblastoma. There
are other issues to be considered in assessing the advantage of
proton compared to conventional radiation therapy. More
detailed information for referrers, patients and families are
available at http://www.specialisedservices.nhs.uk/service/
proton-beam-therapy.
The dilemma faced by radiation oncologists is the safe de-
livery of high dose radiation needed to treat intracranial neo-
plasms with minimal effect on surrounding normal tissues and
avoiding damage to other sensitive structures. This is often
difcult to achieve with photon facility as the doses needed to
arrest tumour progression will inevitably lead to side effects/
toxicities. However, proton has an inherent property that allows
delivery of high dose radiation to tumour while sparing normal
structures. This recognition has lead to development of new
proton beam facilities worldwide. Despite the apparent more cost
of proton compared to photon facilities, an analysis has revealed
a 1.5 cost differential advantage of proton against high technol-
ogy photon treatment facility as long as there are enough patients
to operate an extended schedule e i.e. (6e7days of 16e18 hours)
with at least 2 gantries and a xed horizontal beam. A
FURTHER READING
READING LISTS BOOKS
Keating Robert F, Goodrich James Tait, Roger J. Packer tumours of the
pediatric central nervous system. Thieme, 2001.
Walker David A, Giorgio Perilongo, Punt Jonathan AG, et al. Brain and
spinal tumors of childhood. London: Arnold, 2004.
CHAPTERS
Cohen K, Pollack IF. In: Rudolph CD, Rudolph A, Lister G, First L, Gershon A,
eds. Rudolphs pediatrics. New York: McGraweHill, 2011; 1656e60.
RECENT REVIEWS
Manoranjan B, Provias JP. Congenital brain tumors: diagnostic pitfalls and
therapeutic interventions. J Child Neurol 2011; 26: 599e614.
Gridley DS, Grover RS, Loredo LN, Wroe AJ, Slater JD. Proton beam therapy
for treatment of tumors of the CNS. Expert Rev Neurother 2010; 10:
319e30.
HeadSmart campaign: (http://www.headsmart.org.uk).
Pollack IF, Regina Jakacki RI. Childhood brain tumors: epidemiology, current
management and future directions. Nat Rev Neurol 2011; 7: 495e506.
Northcott PA, Jones DTW, Kool M, Robinson GW, Robertson RJ, Cho Y.
Medulloblastomics: the end of the beginning. Nat Rev Cancer 2012;
12: 818e34.
Proton beam therapy. Guidance for patient referral: http://www.
specialisedservices.nhs.uk/service/proton-beam-therapy.
Wells EM, Gaillard WD, Packer RJ. Pediatric brain tumors and epilepsy.
Semin Pediatr Neurol 2012; 19: 3e8.
Practice points
C
Incidence of brain and spinal tumour have peaked and stabilised
in the 80s and 90s due to improved diagnostic facilities.
C
Current emphasis is on widening awareness among lay public,
general practitioners and paediatrician on presenting features
that will lead to early diagnosis.
C
HeadSmart campaign since June 2011 is showing an improved
trend in the awareness of features of brain tumours in UK and
Ireland.
C
Brain tumours in infants and younger children have different
behaviour, location, histological diagnoses, presenting features,
treatment approach and prognosis when compared to older
children and young adults.
C
Epilepsy can be encountered at any time before or after diagnosis
of a brain tumour, and antineoplastic agents, tumour surgery and
radiotherapy could impact the cause of seizures. Likewise, there
is increasing evidence that certain anti-epileptic drugs have in-
uence in the management of brain tumour.
C
Advanced understanding in biological markers in the last 5e10
year is resulting in better tumour reclassication with impact on
prognosis and therapy strategies.
C
Increasing availability of proton beam radiation facilities world-
wide is enabling the application of high dose radiation to sensi-
tive intracranial sites while avoiding damage to surrounding
normal tissues, thus minimising long term side effects.
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PAEDIATRICS AND CHILD HEALTH 24:4 160 2014 Elsevier Ltd. All rights reserved.