title: Familial gastric cancer: guidelines for diagnosis, treatment and periodic surveillance.
abstract: Hereditary diffuse gastric cancer (HDGC) is a relatively rare disorder, with a mutated CDH1 gene as the only known cause. Carriers of a germline mutation in CDH1 have a lifetime risk of >80% of developing diffuse gastric cancer. As periodic gastric surveillance is of limited value in detecting early stages of HDGC, prophylactic gastrectomy is advised for this patient group. Little is known about other types of familial gastric cancer. The Dutch working group on hereditary gastric cancer has formulated guidelines for various aspects of medical management for families and individuals at high risk of developing gastric cancer, including criteria for referral, classification, diagnostics, and periodic gastric surveillance. These guidelines are not limited to HDGC and are therefore partially complementary to the guidelines on hereditary diffuse gastric cancer of the international gastric cancer linkage consortium (IGCLC 2010). In order to optimize the care and increase the knowledge on hereditary gastric cancer it is important to centralize medical care for these patients. National and international collaboration is warranted to improve the quality of research by increasing the size of study cohorts.
authors.affiliation: Family Cancer Clinic, The Netherlands Cancer Institute, Amsterdam, The Netherlands. i.kluijt@nki.nl
authors.forename: Irma
authors.initials: I
authors.lastname: Kluijt
authors.forename: Rolf H
authors.initials: RH
authors.lastname: Sijmons
authors.forename: Nicoline
authors.initials: N
authors.lastname: Hoogerbrugge
authors.forename: John T
authors.initials: JT
authors.lastname: Plukker
authors.forename: Daphne
authors.initials: D
authors.lastname: de Jong
authors.forename: J Han
authors.initials: JH
authors.lastname: van Krieken
authors.forename: Richard
authors.initials: R
authors.lastname: van Hillegersberg
authors.forename: Marjolijn
authors.initials: M
authors.lastname: Ligtenberg
authors.forename: Eveline
authors.initials: E
authors.lastname: Bleiker
authors.forename: Anemieke
authors.initials: A
authors.lastname: Cats
mesh_terms: D015703:Antigens, CD; D015820:Cadherins / Q000235:genetics*; D003376:Counseling; D005500:Follow-Up Studies; D005820:Genetic Testing; D018095:Germ-Line Mutation; D006801:Humans; D009426:Netherlands; D013274:Stomach Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery*
chemical_list: D015703:Antigens, CD; C513384:CDH1 protein, human; D015820:Cadherins