title: [Hereditary diffuse gastric cancer].
abstract: Some diffuse type gastric cancers are of hereditary origin. Their histological characteristics are poor cell differentiation and the presence of signet-ring cells. The cause is a mutation of the CDH1 gene which is responsible for abnormal E-cadherin. The transmission mode is autosomal dominant. Because of serious prognosis of symptomatic hereditary diffuse gastric cancer (HDGC), the high penetrance of the gene (67% in men and 83% in women) and the young age of onset of these tumors (before the age of 40), a prophylactic gastrectomy is recommended to the mutation carriers. The search for the genetic mutation should be recommended to families corresponding to clinical criteria such as the number of affected family members, degree of relationship and age of onset of these tumors.
authors.affiliation: Service de Chirurgie digestive, Groupe Hospitalier Diaconesses-Croix Saint-Simon, Paris. asezeur@hopital-dcss.org <asezeur@hopital-dcss.org>
authors.forename: Alain
authors.initials: A
authors.lastname: Sezeur
authors.forename: Astrid
authors.initials: A
authors.lastname: Schielke
authors.forename: Lionel
authors.initials: L
authors.lastname: Larue
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Fléjou
mesh_terms: D000230:Adenocarcinoma / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D015820:Cadherins / Q000235:genetics; D018279:Carcinoma, Signet Ring Cell / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control / Q000601:surgery; D005260:Female; D005743:Gastrectomy; D005817:Genetic Counseling; D018095:Germ-Line Mutation / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D011322:Primary Prevention; D011379:Prognosis; D012737:Sex Factors; D013270:Stomach / Q000473:pathology; D013274:Stomach Neoplasms / Q000145:classification / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control / Q000601:surgery; D014944:World Health Organization
chemical_list: D015820:Cadherins