title: Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome.
abstract: OBJECTIVE
  The purpose of this study was the clinical and pathological characterisation of a new autosomal dominant gastric polyposis syndrome, gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS).
  
  
  METHODS
  Case series were examined, documenting GAPPS in three families from Australia, the USA and Canada. The affected families were identified through referral to centralised clinical genetics centres.
  
  
  RESULTS
  The report identifies the clinical and pathological features of this syndrome, including the predominant dysplastic fundic gland polyp histology, the exclusive involvement of the gastric body and fundus, the apparent inverse association with current Helicobacter pylori infection and the autosomal dominant mode of inheritance.
  
  
  CONCLUSIONS
  GAPPS is a unique gastric polyposis syndrome with a significant risk of gastric adenocarcinoma. It is characterised by the autosomal dominant transmission of fundic gland polyposis, including areas of dysplasia or intestinal-type gastric adenocarcinoma, restricted to the proximal stomach, and with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes.
authors.affiliation: Division of Digestive and Liver Diseases, Columbia University, New York, New York, USA.
authors.forename: D L
authors.initials: DL
authors.lastname: Worthley
authors.forename: K D
authors.initials: KD
authors.lastname: Phillips
authors.forename: N
authors.initials: N
authors.lastname: Wayte
authors.forename: K A
authors.initials: KA
authors.lastname: Schrader
authors.forename: S
authors.initials: S
authors.lastname: Healey
authors.forename: P
authors.initials: P
authors.lastname: Kaurah
authors.forename: A
authors.initials: A
authors.lastname: Shulkes
authors.forename: F
authors.initials: F
authors.lastname: Grimpen
authors.forename: A
authors.initials: A
authors.lastname: Clouston
authors.forename: D
authors.initials: D
authors.lastname: Moore
authors.forename: D
authors.initials: D
authors.lastname: Cullen
authors.forename: D
authors.initials: D
authors.lastname: Ormonde
authors.forename: D
authors.initials: D
authors.lastname: Mounkley
authors.forename: X
authors.initials: X
authors.lastname: Wen
authors.forename: N
authors.initials: N
authors.lastname: Lindor
authors.forename: F
authors.initials: F
authors.lastname: Carneiro
authors.forename: D G
authors.initials: DG
authors.lastname: Huntsman
authors.forename: G
authors.initials: G
authors.lastname: Chenevix-Trench
authors.forename: G K
authors.initials: GK
authors.lastname: Suthers
mesh_terms: D000230:Adenocarcinoma / Q000175:diagnosis* / Q000235:genetics / Q000382:microbiology / Q000473:pathology; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005799:Genes, Dominant; D016481:Helicobacter Infections / Q000150:complications; D016480:Helicobacter pylori / Q000302:isolation & purification; D006801:Humans; D008297:Male; D008875:Middle Aged; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis* / Q000235:genetics / Q000382:microbiology / Q000473:pathology; D010375:Pedigree; D011127:Polyps / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D013274:Stomach Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000382:microbiology / Q000473:pathology