world cancer report - iarc

mut = mutant p53
wt = wildtype p53
_ = deletion of p53
tumour sites. They manifest as an abdominal
mass almost exclusively in children
less than 10 years old, with a peak incidence
of 1-4 years. Tumours in very young
children and tumours outside the adrenal
medulla have a better prognosis, and
some lesions regress spontaneously.
Amplification of the N-MYC gene indicates
a poor prognosis.
REFERENCES
36 yr
Low-grade
astrocytoma,
37 yr glioblastoma
1. Lantos PL, Louis DN, Rosenblum MK, Kleihues P
(2002) Tumours of the nervous system. In: Graham DI,
Lantos PL eds, Greenfield's Neuropathology, Seventh
Edition, London, Arnold.
2. Ferlay J, Bray F, Parkin DM, Pisani P (2001) Globocan
47 yr
29 yr
Low-grade Anaplastic
astrocytoma astrocytoma
mut/- in tumour mut/- in tumour
mut/wt in blood
29 yr
mut/wt in blood
8 months
Choroid plexus
carcinoma
mut/- in tumour
wt/wt in blood
wt/wt in blood
3 months
Blue shading = carrier of CGG>TGG mutation in the p53 gene (resulting in a change of amino acid from arginine to tryptophan).
Fig. 5.154 Pedigree of a family with by Li-Fraumeni syndrome, caused by a germline mutation in codon 248
of the p53 tumour suppressor gene. Blood samples of affected family members have a mutation in one allele.
In tumours, the second allele is usually deleted. This family shows a remarkable clustering of brain tumours.
Tumours of peripheral nerves
Most of these tumours develop from
myelin-producing Schwann cells and are
termed neurinomas or schwannomas.
Bilateral acoustic schwannomas are diagnostic
of the inherited neurofibromatosis
type 2. They are benign (WHO Grade I) and
rarely recur after surgical resection.
Neurofibromas and malignant peripheral
2000. Cancer Incidence and Mortality Worldwide (IARC
Cancer Bases No. 5), Lyon, IARCPress.
3. Preston-Martin S, Mack WJ (1996) Neoplasms of the
nervous system. In: Schottenfeld D, Fraumeni, JF eds,
Cancer Epidemiology and Prevention, pp. 1231-1281. New
nerve sheath tumours represent typical
manifestations of the neurofibromatosis
type 1 syndrome.
Meningiomas
These slowly growing, usually benign, neoplasms
develop from arachnoidal cells in
the meninges. They preferentially affect
women, particularly those located in the
spine. Meningiomas do not infiltrate the
brain but may cause symptoms of
intracranial pressure due to compression
of adjacent brain structures (WHO Grade
I). Preferential sites are the cerebral hemispheres.
Meningiomas can often be cured
by surgical resection. Malignant meningiomas
are much less frequent; they may
infiltrate the brain and often recur locally.
Outlook
Although not very frequent, brain tumours
contribute significantly to morbidity, often
affect children and overall have a poor
prognosis. Due to marked resistance to
radiation and chemotherapy, the prognosis
for patients with glioblastomas is very
poor. The majority of patients die within 9-
12 months and less than 3% survive more
than 3 years. Many genetic alterations
involved in the development of nervous
tissue tumours have been identified and
may lead to novel therapeutic approaches,
including gene therapy.
York, Oxford University Press.
4. Kleihues P and Cavenee WK (2000) World Health
Organization Classification of Tumours. Pathology and
Genetics of Tumours of the Nervous System. Lyon,
IARCPress.
Tumours of the nervous system
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