Table 3.

Summary of recommendations for hereditary syndromes with increased gastric cancer risk

Syndrome	HDGC	Lynch syndrome	FAP/aFAP	GAPPS	PJS	JPS	LFS	FIGC
Genes	CDH1, CTNNA1	MLH1, MSH2/EPCAM, MSH6, PMS2	APC	APC promoter 1B	STK11	SMAD4, BMPR1A	TP53	Unknown
Surveillance starting age in years	18–20	30–40	20–25	15	18 (8 for baseline)	12–15	25	40–60 (or 5 years before earliest cancer diagnosis)
Upper endoscopy interval (healthy carriers)	1 year (until prophylactic gastrectomy peformed)	2–4 years	3 months–5 years	1 year (until prophylactic gastrectomy peformed)	2–3 years	1–3 years	2–5 years	1–3 years
Surveillance considerations	Modified Cambridge protocol or Bethesda protocol should be used Inlet patches should be documented and biopsied	Biopsies of the gastric antrum and body should be performed to assess for H. pylori, gastric intestinal metaplasia, and autoimmune gastritis	Surveillance interval should be based on the Spigelman score and/or gastric pathology A baseline upper endoscopy should be performed before 20 years of age if earlier colectomy is planned	–	–	For a clinical diagnosis of JPS without an SMAD4 or BMPR1A PV, the surveillance interval can be increased to 5 years in the absence of gastric polyps	–	–
Surgical considerations	Prophylactic total gastrectomy between 20 and 30 years of age	–	If total gastrectomy is performed, Roux limb should be constructed to allow for continued duodenal surveillance	Prophylactic total gastrectomy by the 30s	–	–	–	–
References	121,124	125	126	126	97,126	97,126	127	114,128

–, no clear guidance established for the condition; HDGC, hereditary diffuse gastric cancer syndrome; FAP, familial adenomatous polyposis; aFAP, attenuated familial adenomatous polyposis; GAPPS, gastric adenocarcinoma and proximal polyposis of the stomach; PJS, Peutz–Jeghers syndrome; JPS, juvenile polyposis syndrome; LFS, Li–Fraumeni syndrome; FIGC, familial intestinal gastric cancer.