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Hereditary diffuse gastric cancer (HDGC) is an inherited genetic syndrome most often caused by an inactivating mutation in the E-cadherin gene (CDH1) located on chromosome 16. [1] Individuals who inherit an inactive copy of the CDH1 gene are at significantly elevated risk for developing stomach cancer .
total gastrectomy, splenectomy for infiltrative gastric cancer. Linitis plastica (sometimes referred to as leather bottle stomach) is a morphological variant of diffuse stomach cancer in which the stomach wall becomes thick and rigid. [1] Linitis plastica is a type of adenocarcinoma and accounts for 3–19% of gastric adenocarcinomas. [1]
Among men, 683 754 cases were diagnosed, accounting for 7.2% of all cancer cases, and among women, stomach cancer was diagnosed in 349 947 cases, accounting for 4.1% of all cancer cases. [96] In 2012, stomach cancer was the fifth most-common cancer with 952,000 cases diagnosed. [16] It is more common both in men and in developing countries.
Hereditary cancer syndromes underlie 5 to 10% of all cancers and there are over 50 identifiable hereditary forms of cancer. [5] Scientific understanding of cancer susceptibility syndromes is actively expanding: additional syndromes are being found, [6] the underlying biology is becoming clearer, and genetic testing is improving detection, treatment, and prevention of cancer syndromes. [7]
Inherited inactivating mutations in CDH1 are associated with hereditary diffuse gastric cancer. Individuals with this condition have up to a 70% lifetime risk of developing diffuse gastric carcinoma, and females with CDH1 mutations have up to a 60% lifetime risk of developing lobular breast cancer. [67]
Gastrointestinal cancer refers to malignant conditions of the gastrointestinal tract (GI tract) and accessory organs of digestion, including the esophagus, stomach, biliary system, pancreas, small intestine, large intestine, rectum and anus. The symptoms relate to the organ affected and can include obstruction (leading to difficulty swallowing ...
Affected individuals have an increased risk of colorectal cancer, precancerous colon polyps and an increased risk of several additional cancers. About 1–2 percent of the population possess a mutated copy of the MUTYH gene, and less than 1 percent of people have the MUTYH-associated polyposis syndrome.
Gastrinoma in the early stages will have signs and symptoms of indigestion [3] or similar to irritable bowel disease (IBD) such as: Hypergastrinemia [3] Refractory or recurrent peptic ulcers involving duodenum [3] Chronic diarrhea [7] [2] [3] Generalized cancer symptoms; Abdominal pain [3] Gastrointestinal bleeding [3] Obstruction of intestine [8]
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