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Pathology of Hereditary Gastric Cancer

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Spotlight on Familial and Hereditary Gastric Cancer

Abstract

Hereditary gastric cancer includes the hereditary diffuse gastric cancer syndrome and a recently described syndrome designated as gastric adenocarcinoma and proximal polyposis of the stomach. Hereditary diffuse gastric cancer is caused by E-cadherin gene (CDH1) alterations and a model of development has been described, encompassing mild non-atrophic gastritis, in situ signet ring cell (diffuse) carcinoma, pagetoid spread of signet ring cells and invasive carcinoma. Gastric adenocarcinoma and proximal polyposis of the stomach is characterized by fundic gland polyposis, with areas of dysplasia or intestinal type gastric cancer, restricted to the proximal stomach, with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes. The genetic cause of gastric adenocarcinoma and proximal polyposis of the stomach has not been identified yet.

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Abbreviations

EGFR:

Epidermal Growth Factor Receptor

FAP:

Familial Adenomatous Polyposis

FDGC:

Familial Diffuse Gastric Cancer

FGC:

Familial Gastric Cancer

FGP:

Fundic Gland Polyposis

GAPPS:

Gastric Adenocarcinoma and Proximal Polyposis of the Stomach

HDGC:

Hereditary Diffuse Gastric Cancer

IGCLC:

International Gastric Cancer Linkage Consortium

MAP:

MYH-Associated Polyposis

PJS:

Peutz–Jeghers Syndrome

RTK:

Receptor Tyrosine Kinase

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Authors and Affiliations

  1. Department of Pathology IPATIMUP, Medical Faculty and Centro Hospitalar de São João,, Porto, Portugal

    Fátima Carneiro M.D., Ph.D.

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  1. Fátima Carneiro M.D., Ph.D.
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Correspondence to Fátima Carneiro M.D., Ph.D. .

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Editors and Affiliations

  1. Dept. of Medical, Surgical Sciences, and Neurosciences, University of Siena, viale Bracci, Siena, 53100, Italy

    Giovanni Corso

  2. Dept. of Medical, Surgical Sciences, and Neurosciences, University of Siena, Via Banchi di Sotto 55, Siena, 53100, Italy

    Franco Roviello

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Carneiro, F. (2013). Pathology of Hereditary Gastric Cancer. In: Corso, G., Roviello, F. (eds) Spotlight on Familial and Hereditary Gastric Cancer. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-6570-2_12

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