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.
Highlights
History is a well-recognized risk factor for gastric cancer, with the most famous example of hereditary transmission of gastric carcinoma being the family of Napoleon Bonaparte
Implications for Practice: the majority of gastric adenocarcinomas are sporadic with many of those related to chronic Helicobacter pylori infection, approximately 10% of the cases show familial aggregation, and a specific hereditary cause is determined in 1%–3% cases.This review describes the molecular genetics, clinical and pathologic features, surveillance guidelines, and preventive measures of common and less common hereditary gastric cancer predisposition syndromes
Clinical and Pathologic Features (Including Associated Other Neoplastic Lesions) In the two series reported to date, the gastric manifestations were evident as early as 10 years of age, and gastric carcinoma was seen at 33 years of age
Summary
History is a well-recognized risk factor for gastric cancer, with the most famous example of hereditary transmission of gastric carcinoma being the family of Napoleon Bonaparte. Clinical and Pathologic Features (Including Associated Other Neoplastic Lesions) In the two series reported to date, the gastric manifestations were evident as early as 10 years of age, and gastric carcinoma was seen at 33 years of age It appears to be more common in females. Endoscopic surveillance is an option offered to carriers of pathogenic mutations who opt not to have gastrectomy, individuals carrying mutations of undetermined significance, and individuals with a strong family history of gastric carcinoma but who test negative for CDH1 mutations [98].The decision to perform genetic testing must be based on the earliest age of cancer onset in the family, but it is recommended that screening be initiated in the late teens or early twenties [97]. Surveillance and Clinical Management XPS patients receive prevention for skin, ocular, and neurologic manifestations, but there are no guidelines for surveillance of gastric carcinoma [145]
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