Hereditary Gastric Cancer
نویسندگان
چکیده
منابع مشابه
Hereditary Gastric Cancer
Hereditary gastric cancer is an autosomal-dominant inherited condition produced by germline mutation in the E-cadherin/CDH1 gene. Criteria to define the syndrome have been proposed and include review of the histopathology and pedigree analysis of any family with an aggregation of gastric cancer cases. Of families with two or more cases of diffuse gastric cancer in firstor second-degree relative...
متن کاملMolecular Pathology of Hereditary Diffuse Gastric Cancer
Hereditary diffuse gastric cancer is a rare, autosomal dominant hereditary cancer syndrome associated with germline mutations in CDH1 in which 60% 80% of affected individuals develop advanced diffuse gastric cancer, many as young adults. At clinical presentation, ~90% of these malignancies represent advanced, surgically incurable disease. As such, pre-symptomatic identification of germline CDH1...
متن کاملHereditary diffuse gastric cancer: One family’s story
Hereditary diffuse gastric cancer (HDGC) is an inherited form of gastric cancer that carries a poor prognosis. Most HDGCs are caused by an autosomal dominant genetic mutation in the CDH1 gene, which carries a 70%-80% lifetime risk of gastric cancer. Given its submucosal origin, endoscopic surveillance is an unreliable means of early detection, and prophylactic gastrectomy is recommended for CDH...
متن کاملGASTRIC CANCER Chromoendoscopic surveillance in hereditary diffuse gastric cancer: an alternative to prophylactic gastrectomy?
Background: Hereditary diffuse gastric cancer (HDGC) is defined by germline mutations in the E-cadherin gene, CDH-1. The first family in which CDH-1 mutations were identified was a large Maori kindred, where lifetime penetrance is 70%. Prophylactic gastrectomy is an unacceptable option for many mutation carriers. The results of annual chromoendoscopic surveillance using the methylene blue/congo...
متن کاملClinical implications of E-cadherin associated hereditary diffuse gastric cancer.
Approximately 1-3% of gastric cancers arise as a result of inherited gastric cancer predisposition syndromes. These may be of the diffuse or intestinal type. Linkage analysis has recently implicated E-cadherin mutations in an estimated 25% of families with an autosomal dominant predisposition to diffuse type gastric cancers. This subset of gastric cancer has been termed hereditary diffuse gastr...
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ژورنال
عنوان ژورنال: JAMA Oncology
سال: 2015
ISSN: 2374-2437
DOI: 10.1001/jamaoncol.2014.187