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Hereditary tumor
syndromes of the nervous system: overview and rare syndromes
Louis DN, von Deimling A
Department of Pathology (Neuropathology) and Neurosurgical Service,
Massachusetts General Hospital, Boston 02114, USA
Hereditary tumor syndromes of the nervous system are a varied group of
conditions that include neurofibromatosis 1, neurofibromatosis 2, tuberous
sclerosis, and von Hippel-Lindau disease, as well as the retinoblastoma
susceptibility, Li-Fraumeni, familial glioma, Turcot, Gorlin, Cowden and
multiple endocrine neoplasia syndromes.
For many of these conditions, the
responsible genes have been localized or identified.
Such studies have
elucidated the genetic basis of both hereditary cancer predisposition and
sporadic nervous system tumors.
The first four hereditary tumors syndromes have
been extensively studied and are discussed in detail in the four subsequent
articles.
The other syndromes have also been subject to both pathological and
molecular genetic inquiries.
In this introductory overview, we discuss the
features common to the hereditary tumor syndromes of the nervous system, and
review some of the rarer conditions.
PMID:
7670655 [PubMed - indexed for MEDLINE]
Source: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7670655
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