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

D. L. Worthley, K. D. Phillips, N. Wayte, K. A. Schrader, S. Healey, P. Kaurah, A. Shulkes, F. Grimpen, A. Clouston, D. Moore, D. Cullen, D. Ormonde, D. Mounkley, X. Wen, Noralane Morey Lindor, F. Carneiro, D. G. Huntsman, G. Chenevix-Trench, Graeme K. Suthers

Research output: Contribution to journalArticle

87 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)774-779
Number of pages6
JournalGut
Volume61
Issue number5
DOIs
StatePublished - May 2012
Externally publishedYes

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Stomach
Adenocarcinoma
Gastric Fundus
Gastrointestinal Neoplasms
Helicobacter Infections
Polyps
Helicobacter pylori
Canada
Histology
Referral and Consultation

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Worthley, D. L., Phillips, K. D., Wayte, N., Schrader, K. A., Healey, S., Kaurah, P., ... Suthers, G. K. (2012). Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome. Gut, 61(5), 774-779. https://doi.org/10.1136/gutjnl-2011-300348

Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) : A new autosomal dominant syndrome. / Worthley, D. L.; Phillips, K. D.; Wayte, N.; Schrader, K. A.; Healey, S.; Kaurah, P.; Shulkes, A.; Grimpen, F.; Clouston, A.; Moore, D.; Cullen, D.; Ormonde, D.; Mounkley, D.; Wen, X.; Lindor, Noralane Morey; Carneiro, F.; Huntsman, D. G.; Chenevix-Trench, G.; Suthers, Graeme K.

In: Gut, Vol. 61, No. 5, 05.2012, p. 774-779.

Research output: Contribution to journalArticle

Worthley, DL, Phillips, KD, Wayte, N, Schrader, KA, Healey, S, Kaurah, P, Shulkes, A, Grimpen, F, Clouston, A, Moore, D, Cullen, D, Ormonde, D, Mounkley, D, Wen, X, Lindor, NM, Carneiro, F, Huntsman, DG, Chenevix-Trench, G & Suthers, GK 2012, 'Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome', Gut, vol. 61, no. 5, pp. 774-779. https://doi.org/10.1136/gutjnl-2011-300348
Worthley DL, Phillips KD, Wayte N, Schrader KA, Healey S, Kaurah P et al. Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome. Gut. 2012 May;61(5):774-779. https://doi.org/10.1136/gutjnl-2011-300348
Worthley, D. L. ; Phillips, K. D. ; Wayte, N. ; Schrader, K. A. ; Healey, S. ; Kaurah, P. ; Shulkes, A. ; Grimpen, F. ; Clouston, A. ; Moore, D. ; Cullen, D. ; Ormonde, D. ; Mounkley, D. ; Wen, X. ; Lindor, Noralane Morey ; Carneiro, F. ; Huntsman, D. G. ; Chenevix-Trench, G. ; Suthers, Graeme K. / Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) : A new autosomal dominant syndrome. In: Gut. 2012 ; Vol. 61, No. 5. pp. 774-779.
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