Gastric adenocarcinoma and proximal polyposis of the stomach

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Gastric adenocarcinoma and proximal polyposis of the stomach, abbreviated GAPPS, is a rare syndrome characterized by fundic gland polyps and gastric adenocarcinoma.[1][2]

General

  • Very rare.
  • APC promotor IB mutations.[1]
  • Autosomal dominant inheritance.[1]

Criteria

Features - all required:[1]

  • Polyps of stomach - body and fundus only.
    • >100 polyps in index case or >30 polyps in first degree relative.
    • Histology predominantly fundic gland polyps - some with dysplasia or adenocarcinoma in background.
  • No colorectal or duodenal polyposis.
  • Exclusion of other heritable polyposis syndrome involving the stomach, proton pump inhibitor (PPI) use.

See also

References

  1. 1.0 1.1 1.2 1.3 Rudloff, U. (2018). "Gastric adenocarcinoma and proximal polyposis of the stomach: diagnosis and clinical perspectives.". Clin Exp Gastroenterol 11: 447-459. doi:10.2147/CEG.S163227. PMID 30584346.
  2. Worthley, DL.; Phillips, KD.; Wayte, N.; Schrader, KA.; Healey, S.; Kaurah, P.; Shulkes, A.; Grimpen, F. et al. (May 2012). "Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome.". Gut 61 (5): 774-9. doi:10.1136/gutjnl-2011-300348. PMID 21813476.