title: A RAND/UCLA appropriateness study of the management of familial gastric cancer.
abstract: BACKGROUND
  Hereditary diffuse gastric cancer (HDGC) represents a minority of gastric cancer (GC) cases. The goal of this study is to use a RAND/University of California Los Angeles (UCLA) appropriateness methodology to examine indications for genetic referral, CDH1 testing, and consideration of prophylactic total gastrectomy (PTG).
  
  
  METHODS
  A multidisciplinary expert panel of 16 physicians from six countries scored 47 scenarios. Appropriateness of scenarios was scored from 1 (highly inappropriate) to 9 (highly appropriate). Median appropriateness scores (AS) of 1-3 were considered inappropriate, 4-6 uncertain, and 7-9 appropriate. Agreement was reached when 12 of 16 panelists scored the statement similarly. Appropriate scenarios agreed upon were subsequently scored for necessity.
  
  
  RESULTS
  The panel felt that patients with family history of diffuse gastric cancer (DGC), lobular breast cancer, or multiple family members with GC should be referred for genetic assessment and multidisciplinary decision-making. The panel felt that it is appropriate for patients with DGC to have CDH1 mutation testing in a family with (1) ≥2 cases of GC, with at least one case of DGC diagnosed before age of 50 years; (2) ≥3 cases of GC diagnosed at any age, one or more of which is DGC; (3) a patient diagnosed with DGC and lobular breast carcinoma; or (4) patients diagnosed with DGC under age of 35 years. The panel felt that PTG should be offered to CDH1 mutation carriers 20 years or older.
  
  
  CONCLUSIONS
  Identification of genetic mutations in patients at risk for hereditary GC is important, and criteria for testing are suggested.
authors.affiliation: Sunnybrook Research Institute, Toronto, ON, Canada.
authors.forename: Matthew
authors.initials: M
authors.lastname: Dixon
authors.forename: Rajini
authors.initials: R
authors.lastname: Seevaratnam
authors.forename: Debrah
authors.initials: D
authors.lastname: Wirtzfeld
authors.forename: Robin
authors.initials: R
authors.lastname: McLeod
authors.forename: Lucy
authors.initials: L
authors.lastname: Helyer
authors.forename: Calvin
authors.initials: C
authors.lastname: Law
authors.forename: Carol
authors.initials: C
authors.lastname: Swallow
authors.forename: Lawrence
authors.initials: L
authors.lastname: Paszat
authors.forename: Alina
authors.initials: A
authors.lastname: Bocicariu
authors.forename: Roberta
authors.initials: R
authors.lastname: Cardoso
authors.forename: Alyson
authors.initials: A
authors.lastname: Mahar
authors.forename: Tanios
authors.initials: T
authors.lastname: Bekaii-Saab
authors.forename: Ian
authors.initials: I
authors.lastname: Chau
authors.forename: Neal
authors.initials: N
authors.lastname: Church
authors.forename: Daniel
authors.initials: D
authors.lastname: Coit
authors.forename: Christopher H
authors.initials: CH
authors.lastname: Crane
authors.forename: Craig
authors.initials: C
authors.lastname: Earle
authors.forename: Paul
authors.initials: P
authors.lastname: Mansfield
authors.forename: Norman
authors.initials: N
authors.lastname: Marcon
authors.forename: Thomas
authors.initials: T
authors.lastname: Miner
authors.forename: Sung Hoon
authors.initials: SH
authors.lastname: Noh
authors.forename: Geoff
authors.initials: G
authors.lastname: Porter
authors.forename: Mitchell C
authors.initials: MC
authors.lastname: Posner
authors.forename: Vivek
authors.initials: V
authors.lastname: Prachand
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sano
authors.forename: Cornelis J H
authors.initials: CJ
authors.lastname: Van de Velde
authors.forename: Sandra
authors.initials: S
authors.lastname: Wong
authors.forename: Natalie
authors.initials: N
authors.lastname: Coburn
mesh_terms: D000328:Adult; D015703:Antigens, CD; D001943:Breast Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D015820:Cadherins / Q000235:genetics*; D018275:Carcinoma, Lobular / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D019468:Disease Management; D005190:Family; D005260:Female; D005743:Gastrectomy; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D011379:Prognosis; D013274:Stomach Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery
chemical_list: D015703:Antigens, CD; C513384:CDH1 protein, human; D015820:Cadherins