title: Familial Gastric Cancers.
abstract: Although the majority of gastric carcinomas are sporadic, approximately 10% show familial aggregation, and a hereditary cause is determined in 1%-3% cases. Of these, hereditary diffuse gastric cancer is the most recognized predisposition syndrome. Although rare, the less commonly known syndromes also confer a markedly increased risk for development of gastric cancer. Identification and characterization of these syndromes require a multidisciplinary effort involving oncologists, surgeons, genetic counselors, biologists, and pathologists. This article reviews the molecular genetics, clinical and pathologic features, surveillance guidelines, and preventive measures of common and less common hereditary gastric cancer predisposition syndromes.
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Namrata
authors.initials: N
authors.lastname: Setia
authors.affiliation: Department of Hematology/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Jeffrey W
authors.initials: JW
authors.lastname: Clark
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Dan G
authors.initials: DG
authors.lastname: Duda
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Theodore S
authors.initials: TS
authors.lastname: Hong
authors.affiliation: Department of Hematology/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Eunice L
authors.initials: EL
authors.lastname: Kwak
authors.affiliation: Department of Surgical Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: John T
authors.initials: JT
authors.lastname: Mullen
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA glauwers@partners.org.
authors.forename: Gregory Y
authors.initials: GY
authors.lastname: Lauwers
mesh_terms: D000230:Adenocarcinoma / Q000235:genetics / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics* / Q000517:prevention & control; D011127:Polyps / Q000235:genetics / Q000473:pathology; D013272:Stomach Diseases / Q000235:genetics / Q000473:pathology; D013274:Stomach Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000517:prevention & control
keywords: Cancer; Familial; Hereditary; Stomach; Syndromic