Familial gastric carcinoma
作者： Fátima Carneiro , Xiaogang Wen , Raquel Seruca , Carla Oliveira
DOI: 10.1016/J.MPDHP.2014.03.010
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摘要: Abstract Most cases of gastric cancer are sporadic and familial clustering is observed in about 10% cases. Hereditary accounts for a very low percentage (1–3%), encompassing: hereditary diffuse (HDGC) adenocarcinoma proximal polyposis the stomach (GAPPS). Furthermore, can develop setting other syndromes such as Li–Fraumeni syndrome, Familial Adenomatous Polyposis, Peutz–Jeghers Lynch MUTYH-associated adenomatous polyposis, Juvenile Polyposis Cowden syndrome. HDGC caused by alterations CDH1 gene that encodes e-cadherin model development encompasses non-atrophic gastritis, situ signet ring cell carcinoma, pagetoid spread cells invasive carcinoma. GAPPS characterized fundic gland with areas dysplasia or intestinal-type cancer, without evidence colorectal heritable gastrointestinal syndromes. The genetic cause has not been identified yet.
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