Wilne, Sophie, Collier, Jacqueline, Kennedy, Colin R., Koller, Karin, Grundy, Richard and Walker, David
The presentation of childhood CNS tumours: a systematic review and meta-analysis
The Lancet Oncology, 8, (8), . (doi:10.1016/S1470-2045(07)70207-3). (PMID:17644483).
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Background Suspicion of a CNS tumour is classically raised by symptoms of raised intracranial pressure, focal defi cits
(including seizures), or papilloedema. Development of guidelines is needed for the identifi cation and referral of
children who might have a CNS tumour. We did a systematic literature review and meta-analysis to identify the
clinical presentation of childhood CNS tumours to provide evidence to support the development of guidelines to
assist with the identifi cation and referral for imaging of children who might have a central nervous system tumour.
Methods Medline, Embase, and PubMed were searched for cohort studies and case series in children, published
between January, 1991, and August, 2005, detailing the symptoms and signs at diagnosis of a CNS tumour.
Findings 74 papers (n=4171) met the inclusion criteria. 56 symptoms and signs at diagnosis were identifi ed, ranked by
frequency, and clustered according to age, anatomical criteria, and genetic criteria. The most frequent symptoms and
signs at diagnosis were: headache (33%), nausea and vomiting (32%), abnormalities of gait and coordination (27%),
and papilloedema (13%) for intracranial tumours; macrocephaly (41%), nausea and vomiting (30%), irritability (24%),
and lethargy (21%) for children aged under 4 years with intracranial tumours; reduced visual acuity (41%),
exophthalmia (16%), and optic atrophy (15%) for children with an intracranial tumour and neurofi bromatosis; nausea
and vomiting (75%), headache (67%), abnormal gait and coordination (60%), and papilloedema (34%) for posterior
fossa tumours; unspecifi ed symptoms and signs of raised intracranial pressure (47%), seizures (38%), and
papilloedema (21%) for supratentorial tumours; headache (49%), abnormal eye movements (21%), squint (21%), and
nausea and vomiting (19%) for central brain tumours; abnormal gait and coordination (78%), cranial nerve palsies
(52%), pyramidal signs (33%), headache (23%), and squint (19%) for brainstem tumours; and back pain (67%),
abnormalities of gait and coordination (42%), spinal deformity (39%), focal weakness (21%), and sphincter disturbance
(20%) for spinal-cord tumours. Other features noted were weight loss, growth failure, and precocious puberty.
Symptoms of raised intracranial pressure were absent in more than half of children with brain tumours. Other
neurological features were heterogeneous and related to tumour location.
Interpretation Apart from raised intracranial pressure, motor and visual system abnormalities, weight loss,
macrocephaly, growth failure, and precocious puberty also suggest presence of an intracranial tumour. Children with
signs and symptoms that could result from a CNS tumour need a thorough visual and motor system examination and
an assessment of growth and pubertal status. Occurrence of multiple symptoms and signs should alert clinicians to
possible CNS tumours.
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