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Hereditary Gastric Cancer: A New Syndrome

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Gastric Cancer: the 25-year R-Evolution

Part of the book series: Updates in Surgery ((UPDATESSURG))

Abstract

Hereditary diffuse gastric cancer is an autosomal dominant syndrome associated with E-cadherin protein gene (CDH1) germline mutations. Clinical criteria for genetic screening were revised in 2020 by the International Gastric Cancer Linkage Consortium. About 40% of families fulfilling clinical criteria for this inherited disease present deleterious CDH1 germline mutations. Lobular breast cancer is a neoplastic condition associated with the hereditary diffuse gastric cancer syndrome. E-cadherin constitutional mutations have been described in both settings, in gastric and breast cancers. The only life-saving procedure is prophylactic total gastrectomy for deleterious CDH1 germline mutant carriers. In this chapter we will describe the multidisciplinary clinical management of this complex inherited cancer predisposition.

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Authors and Affiliations

  1. Division of Breast Surgery, European Institute of Oncology IRCCS, Milan, Italy

    Giovanni Corso, Francesca Magnoni & Antonia Girardi

  2. Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy

    Giovanni Corso

  3. Division of Endoscopy, European Institute of Oncology IRCCS, Milan, Italy

    Cristina Trovato

  4. i3S—Instituto de Investigação e Inovação em Saúde, Universidade do Porto, Porto, Portugal

    Raquel Seruca & Joana Figueiredo

  5. Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal

    Raquel Seruca & Joana Figueiredo

  6. Unit of General Surgery and Surgical Oncology, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy

    Franco Roviello

Authors
  1. Giovanni Corso
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  2. Francesca Magnoni
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  3. Cristina Trovato
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  4. Antonia Girardi
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  5. Raquel Seruca
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  6. Joana Figueiredo
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  7. Franco Roviello
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Correspondence to Giovanni Corso .

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Editors and Affiliations

  1. Division of General and Upper Gastrointestinal Surgery, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, Verona, Italy

    Giovanni de Manzoni

  2. Unit of General Surgery and Surgical Oncology, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy

    Franco Roviello

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Corso, G. et al. (2022). Hereditary Gastric Cancer: A New Syndrome. In: de Manzoni, G., Roviello, F. (eds) Gastric Cancer: the 25-year R-Evolution. Updates in Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-73158-8_5

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  • DOI: https://doi.org/10.1007/978-3-030-73158-8_5

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