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Genetics, prognosis and
therapy of central nervous system tumors
Watkins D, Rouleau GA
Division of Medical Genetics, McGill University, Montreal,
Canada
Tumors of the central nervous system (CNS) are common causes of morbidity
and mortality.
These tumors can occur sporadically or in individuals with
genetic disorders predisposing to cancer development.
Such syndromes include
neurofibromatosis type 2, neurofibromatosis type 1, Li-Fraumeni syndrome, as
well as von Hippel-Lindau disease, tuberous sclerosis, and Turcot syndrome.
There may also be familial syndromes resulting in glioma or meningioma alone,
but these are not well understood.
Development of sporadic gliomas is
accompanied by a number of molecular genetic alterations, including activation
of dominant oncogenes and inactivation of tumor suppressor genes.
Some of these
alterations may be associated with progression of gliomas to their most
malignant form, glioblastoma multiforme.
However, at this time molecular genetic
analysis of gliomas does not provide better prognosis than histopathological
staging.
Recently, experimental treatments of gliomas in rodents, using gene
therapy, have been reported.
Results of these studies have been promising, and
these techniques may represent a future direction for therapy in humans.
PMID: 8025896, UI: 94298023
Source: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8025896
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