Article

Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome.

Division of Digestive and Liver Diseases, Columbia University, New York, New York, USA.
Gut (impact factor: 10.11). 08/2011; 61(5):774-9. DOI:10.1136/gutjnl-2011-300348 pp.774-9
Source: PubMed

ABSTRACT 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).
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.
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.
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.

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Keywords

affected families
 
apparent inverse association
 
autosomal dominant mode
 
autosomal dominant transmission
 
centralised clinical genetics centres
 
colorectal
 
current Helicobacter pylori infection
 
duodenal polyposis
 
families
 
fundic gland polyposis
 
gastric adenocarcinoma
 
gastric body
 
heritable gastrointestinal cancer syndromes
 
intestinal-type gastric adenocarcinoma
 
new autosomal dominant gastric polyposis syndrome
 
predominant dysplastic fundic gland polyp histology
 
proximal polyposis
 
proximal stomach
 
significant risk
 
unique gastric polyposis syndrome