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PubMed · 17075480

[Hereditary diffuse gastric cancer].

Alain Sezeur·Astrid Schielke·Lionel Larue·Jean-François Fléjou
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Abstract

Some diffuse type gastric cancers are of hereditary origin. Their histological characteristics are poor cell differentiation and the presence of signet-ring cells. The cause is a mutation of the CDH1 gene which is responsible for abnormal E-cadherin. The transmission mode is autosomal dominant. Because of serious prognosis of symptomatic hereditary diffuse gastric cancer (HDGC), the high penetrance of the gene (67% in men and 83% in women) and the young age of onset of these tumors (before the age of 40), a prophylactic gastrectomy is recommended to the mutation carriers. The search for the genetic mutation should be recommended to families corresponding to clinical criteria such as the number of affected family members, degree of relationship and age of onset of these tumors.

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AdenocarcinomaAdolescentAdultAge FactorsCadherinsCarcinoma, Signet Ring CellFemaleGastrectomyGenetic CounselingGerm-Line MutationHeterozygoteHumansMaleMiddle AgedNeoplastic Syndromes, HereditaryPrimary PreventionPrognosisSex FactorsStomachStomach NeoplasmsWorld Health OrganizationComparative StudyEnglish AbstractJournal ArticleReview

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Alain Sezeur, Astrid Schielke, Lionel Larue, Jean-François Fléjou. 2006. [Hereditary diffuse gastric cancer].. https://doi.org/10.1016/s0399-8320(06)73513-8

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