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In the last decade, many of the genes responsible for these typically autosomal dominant familial tumor syndromes have been identified. Additionally, our understanding of the mechanisms of carcinogenesis in these syndromes has increased, allowing for more targeted therapies for these patients as well as those with sporadic cancers. Because these patients present a unique set of issues regarding diagnosis and neurooncological management, the most common familial cancer syndromes involving the nervous system are reviewed: neurofibromatosis type 1 and 2; tuberous sclerosis complex; von Hippel Lindau, Li-Fraumeni, Gorlin, and Turcot syndrome.<\/jats:p>","DOI":"10.1177\/0883073809337539","type":"journal-article","created":{"date-parts":[[2009,12,2]],"date-time":"2009-12-02T13:39:38Z","timestamp":1259761178000},"page":"1526-1535","source":"Crossref","is-referenced-by-count":46,"title":["Neurooncology of Familial Cancer Syndromes"],"prefix":"10.1177","volume":"24","author":[{"given":"Andreas F.","family":"Hottinger","sequence":"first","affiliation":[{"name":"H\u00f4pitaux Universitaire Genevois and Department of Oncology, University of Geneva, Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasmin","family":"Khakoo","sequence":"additional","affiliation":[{"name":"Memorial 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