Incidence of childhood CNS
tumours in Britain and variation in rates by definition of malignant behaviour:
population-based study
·
Aruna Chakrabarty, ·
Tom Kenny and ·
Paul Chumas
BMC Cancer201919:139
© The Author(s). 2019
·
Received: 18 September 2017 ·
Accepted: 1 February 2019
·
Published: 11 February 2019
Background
Intracranial and intraspinal tumours are the most numerous solid tumours in
children. Some recently defined subtypes are relatively frequent in childhood.
Many cancer registries routinely ascertain CNS tumours of all behaviours, while
others only cover malignant neoplasms. Some behaviour codes have changed
between revisions of the International Classification of Diseases for Oncology,
including pilocytic astrocytoma, downgraded to uncertain behaviour in ICD-O-3.
Methods
We used data from the population-based National Registry of Childhood
Tumours, which routinely included non-malignant CNS tumours, to document the
occurrence of CNS tumours among children aged < 15 years in Great Britain
during 2001–2010 and to document the descriptive epidemiology of childhood CNS
tumours over the 40-year period 1971–2010, during which several new entities
were accommodated in successive editions of the WHO Classification and
revisions of ICD-O. Eligible cases were all those with a diagnosis included in
Groups III (CNS tumours) and Xa (CNS germ-cell tumours) of the International
Classification of Childhood Cancer, Third Edition. The population at risk was
derived from annual mid-year estimates by sex and single year of age compiled
by the Office for National Statistics and its predecessors. Incidence rates
were calculated for age groups 0, 1–4, 5–9 and 10–14 years, and
age-standardised rates were calculated using the weights of the world standard
population.
Results
Age-standardised incidence in 2001–10 was 40.1 per million. Astrocytomas
accounted for 41%, embryonal tumours for 17%, other gliomas for 10%,
ependymomas for 7%, rarer subtypes for 20% and unspecified tumours for 5%.
Incidence of tumours classified as malignant and non-malignant by ICD-O-3
increased by 30 and 137% respectively between 1971-75 and 2006–10.
Conclusions
Total incidence was similar to that in other large western countries.
Deficits of some, predominantly low-grade, tumours or differences in their age
distribution compared with the United States and Nordic countries are
compatible with delayed diagnosis. Complete registration regardless of tumour
behaviour is essential for assessing burden of disease and changes over time.
This is particularly important for pilocytic astrocytoma, because of its recent
downgrading to non-malignant and time trends in the proportion of astrocytomas
with specified subtype.
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