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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 .
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]
Primary gastric lymphoma (lymphoma that originates in the stomach itself) [1] is an uncommon condition, accounting for less than 15% of gastric malignancies and about 2% of all lymphomas. However, the stomach is a very common extranodal site for lymphomas (lymphomas originate elsewhere and metastasise to the stomach). [ 2 ]
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 ]
Stomach cancer, also known as gastric cancer, is a malignant tumor of the stomach. It's a cancer that develops from the lining of the stomach . [ 10 ] Most cases of stomach cancers are gastric carcinomas , which can be divided into a number of subtypes, including gastric adenocarcinomas . [ 2 ]
A 2006 review stated that Reed's Syndrome often is the leading cause of renal cancer between ages 30–50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974–1976 and 1995–2000, from 52% to 64%. [18]
Cancer of the stomach, also called gastric cancer, is the fourth-most-common type of cancer and the second-highest cause of cancer death globally. [2] Eastern Asia (China, Japan, Korea, Mongolia) is a high-risk area for gastric cancer, and North America, Australia, New Zealand and western and northern Africa are areas with low risk. [5]