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In a little over a decade, we have seen a tremendous increase in knowledge of the primary genetic basis of many of the familial cancer syndromes that involve the nervous system, syndromes that are mostly inherited as autosomal dominant traits. In this review, we discuss the most recent findings on the genetic basis of hereditary nervous system tumors. The identification of genes associated with familial cancer syndromes has in some families enabled a \u201cmolecular diagnosis\u201d that complements clinical assessment and allows directed cancer surveillance for those individuals determined to be at\u2010risk for disease. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.c.30022","type":"journal-article","created":{"date-parts":[[2004,7,6]],"date-time":"2004-07-06T17:01:35Z","timestamp":1089133295000},"page":"74-84","source":"Crossref","is-referenced-by-count":61,"title":["Genetic insights into familial tumors of the nervous 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