title: Familial gastric cancer: overview and guidelines for management.
abstract: Families with autosomal dominant inherited predisposition to gastric cancer have been described. More recently, germline E-cadherin/CDH1 mutations have been identified in hereditary diffuse gastric cancer kindred. The need to have protocols to manage and counsel these families in the clinic led a group of geneticists, gastroenterologists, surgeons, oncologists, pathologists, and molecular biologists to convene a workshop to produce consensus statements and guidelines for familial gastric cancer. Review of the available cancer pathology from people belonging to families with documented germline E-cadherin/CDH1 mutations confirmed that the gastric cancers were all of the diffuse type. Criteria to define the different types of familial gastric cancer syndromes were agreed. Foremost among these criteria was that review of histopathology should be part of the evaluation of any family with aggregation of gastric cancer cases. Guidelines for genetic testing and counselling in hereditary diffuse gastric cancer were produced. Finally, a proposed strategy for clinical management in families with high penetrance autosomal dominant predisposition to gastric cancer was defined.
authors.affiliation: Department of Oncology, Cambridge Institute for Medical Research and Strangeways Research Laboratories, University of Cambridge, Addenbrooke's Hospital, Cambridge CB2 2XY, UK.
authors.forename: C
authors.initials: C
authors.lastname: Caldas
authors.forename: F
authors.initials: F
authors.lastname: Carneiro
authors.forename: H T
authors.initials: HT
authors.lastname: Lynch
authors.forename: J
authors.initials: J
authors.lastname: Yokota
authors.forename: G L
authors.initials: GL
authors.lastname: Wiesner
authors.forename: S M
authors.initials: SM
authors.lastname: Powell
authors.forename: F R
authors.initials: FR
authors.lastname: Lewis
authors.forename: D G
authors.initials: DG
authors.lastname: Huntsman
authors.forename: P D
authors.initials: PD
authors.lastname: Pharoah
authors.forename: J A
authors.initials: JA
authors.lastname: Jankowski
authors.forename: P
authors.initials: P
authors.lastname: MacLeod
authors.forename: H
authors.initials: H
authors.lastname: Vogelsang
authors.forename: G
authors.initials: G
authors.lastname: Keller
authors.forename: K G
authors.initials: KG
authors.lastname: Park
authors.forename: F M
authors.initials: FM
authors.lastname: Richards
authors.forename: E R
authors.initials: ER
authors.lastname: Maher
authors.forename: S A
authors.initials: SA
authors.lastname: Gayther
authors.forename: C
authors.initials: C
authors.lastname: Oliveira
authors.forename: N
authors.initials: N
authors.lastname: Grehan
authors.forename: D
authors.initials: D
authors.lastname: Wight
authors.forename: R
authors.initials: R
authors.lastname: Seruca
authors.forename: F
authors.initials: F
authors.lastname: Roviello
authors.forename: B A
authors.initials: BA
authors.lastname: Ponder
authors.forename: C E
authors.initials: CE
authors.lastname: Jackson
mesh_terms: D015820:Cadherins / Q000235:genetics*; D005743:Gastrectomy; D005817:Genetic Counseling; D020022:Genetic Predisposition to Disease; D017408:Guidelines as Topic; D006801:Humans; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery / Q000628:therapy
chemical_list: D015820:Cadherins