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Hereditary Diffuse Gastric Cancer

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Gastric Cancer

Abstract

Hereditary diffuse gastric cancer (HDGC) is a rare form of gastric cancer composing only about 1–3 % of gastric cancers. It is caused by a mutation in CDH1 that is a subtype of the E-cadherin gene. It is inherited as an autosomal dominant trait. It has incomplete penetrance and between 70–90 % of patients who inherit the defective gene will develop gastric cancer. Approximately 40 % of women who have the mutation will also develop lobular breast cancer. HDGC causes a diffuse signet ring cell adenocarcinoma of the stomach and requires a total gastrectomy. Patients who are diagnosed by screening are usually cured, while those who wait for symptoms are not. Gastrectomy is advised in carriers approximately 5 years before the youngest family member known to have developed gastric cancer. In our experience, a high proportion (> 90 %) of patients who meet these criteria are found to have diffuse gastric cancer. The cancers are more common in the proximal stomach and cardia, but they also occur in the antrum.

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Author information

Authors and Affiliations

  1. Med Center Line, Surgery – General Surgery, Stanford University, Stanford, CA, USA

    George Poultsides MD

  2. Surgical Oncology and General Surgery, Stanford University, 300 Pasteur Dr., H3591, 94305-5641, Stanford, CA, USA

    Jeffrey A. Norton MD

Authors
  1. George Poultsides MD
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  2. Jeffrey A. Norton MD
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Corresponding author

Correspondence to Jeffrey A. Norton MD .

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Editors and Affiliations

  1. Gastric and Mixed Tumor Service, Memorial Sloan-Kettering Cancer Center, New York, New York, USA

    Vivian E. Strong

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© 2015 Springer International Publishing Switzerland

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Poultsides, G., Norton, J. (2015). Hereditary Diffuse Gastric Cancer. In: Strong, V. (eds) Gastric Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-15826-6_18

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  • DOI: https://doi.org/10.1007/978-3-319-15826-6_18

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-15825-9

  • Online ISBN: 978-3-319-15826-6

  • eBook Packages: MedicineMedicine (R0)

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