Table 2.

Hereditary cancer syndromes.

Hereditary cancer syndrome	Responsible gene(s)	Inheritance	Lifetime risk of gastric cancer	Other malignancies
Lynch syndrome (LS)	MLH1, MSH2, MSH6, PMS2, EPCAM	AD	1–13%	Uterus, ovary, hepatobiliary, urological, pancreas, brain, small bowel (3–25)
Li-Fraumeni syndrome (LFS)	TP53	AD	<3%	Sarcoma, breast, brain, adrenal cortical (2, 6)
Peutz-Jeghers syndrome (PJS)	STK11	AD	29%	Breast, pancreas, small bowel, lung, ovary, testis, cervix (27)
Familial adenomatous polyposis (FAP)	APC	AD	~0.6%	Thyroid, duodenum, small bowel, brain (28)
Juvenile polyposis syndrome (JPS)	SMAD4, BMPR1A	AD	30% in those with SMAD4 mutations	Small bowel, pancreas (2, 9, 30)
MUTYH-associated adenomatous polyposis (MAP)	MUTYH	AR	No statistically significant data available	Thyroid, duodenum (1, 6)
Hereditary breast and ovarian cancer syndrome (HBOC)	BRCA1, BRCA2	AD	Undefined	Breast, ovary, pancreas, melanoma (31–33)

AD, autosomal dominant; AR, autosomal recessive.