Nervous system tumors represent unique neoplasms that arise within the central and peripheral nervous system. While the vast majority of nervous system neoplasm occur sporadically, most of the adult and pediatric forms have a hereditary equivalent. 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 "molecular diagnosis" that complements clinical assessment and allows directed cancer surveillance for those individuals determined to be at-risk for disease.

Genetic insights into familial tumors of the nervous system / German Melean; Roberta Sestini; Franco Ammannati; Laura Papi. - In: AMERICAN JOURNAL OF MEDICAL GENETICS. PART C, SEMINARS IN MEDICAL GENETICS. - ISSN 1552-4868. - STAMPA. - 129C:(2004), pp. 74-84. [10.1002/ajmg.c.30022]

Genetic insights into familial tumors of the nervous system.

SESTINI, ROBERTA;AMMANNATI, FRANCO;PAPI, LAURA
2004

Abstract

Nervous system tumors represent unique neoplasms that arise within the central and peripheral nervous system. While the vast majority of nervous system neoplasm occur sporadically, most of the adult and pediatric forms have a hereditary equivalent. 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 "molecular diagnosis" that complements clinical assessment and allows directed cancer surveillance for those individuals determined to be at-risk for disease.
2004
129C
74
84
German Melean; Roberta Sestini; Franco Ammannati; Laura Papi
File in questo prodotto:
File Dimensione Formato  
AmJ Med Genet.pdf

Accesso chiuso

Tipologia: Versione finale referata (Postprint, Accepted manuscript)
Licenza: Tutti i diritti riservati
Dimensione 135.11 kB
Formato Adobe PDF
135.11 kB Adobe PDF   Richiedi una copia

I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/331047
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 60
  • ???jsp.display-item.citation.isi??? 42
social impact