Difference between revisions of "Gastric adenocarcinoma and proximal polyposis of the stomach"
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==Criteria== | ==Criteria== | ||
Features - all required:<ref name=pmid30584346/> | Features - all required:<ref name=pmid30584346/> | ||
*Polyps of stomach | *Polyps of stomach - body and fundus only. | ||
**>100 polyps in index case ''or'' >30 polyps in first degree relative. | **>100 polyps in index case ''or'' >30 polyps in first degree relative. | ||
**Histology predominantly [[fundic gland polyp]]s - some with dysplasia or adenocarcinoma. | **Histology predominantly [[fundic gland polyp]]s - some with dysplasia or adenocarcinoma in background. | ||
*No colorectal or duodenal polyposis. | *No colorectal or duodenal polyposis. | ||
*Autosomal dominant inheritance. | *Autosomal dominant inheritance. |
Revision as of 16:47, 16 September 2019
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 - 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.
- Autosomal dominant inheritance.
- Exclusion of other heritable polyposis syndrome involving the stomach, PPI use.
See also
References
- ↑ 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.
- ↑ 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.