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]

Criteria

Features - all required:[1]

  • Polyps of stomach in 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.
  • No colorectal or duodenal polyposis.
  • Autosomal dominant inheritance.
  • Exclusion of other heritable polyposis syndrome involving the stomach, PPI use.

See also

References

  1. 1.0 1.1 1.2 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.