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Familial Cancer

, Volume 11, Issue 3, pp 363–369 | Cite as

Familial gastric cancer: guidelines for diagnosis, treatment and periodic surveillance

  • Irma Kluijt
  • Rolf H. Sijmons
  • Nicoline Hoogerbrugge
  • John T. Plukker
  • Daphne de Jong
  • J. Han van Krieken
  • Richard van Hillegersberg
  • Marjolijn Ligtenberg
  • Eveline Bleiker
  • Anemieke Cats
Original Article

Abstract

Hereditary diffuse gastric cancer (HDGC) is a relatively rare disorder, with a mutated CDH1 gene as the only known cause. Carriers of a germline mutation in CDH1 have a lifetime risk of >80% of developing diffuse gastric cancer. As periodic gastric surveillance is of limited value in detecting early stages of HDGC, prophylactic gastrectomy is advised for this patient group. Little is known about other types of familial gastric cancer. The Dutch working group on hereditary gastric cancer has formulated guidelines for various aspects of medical management for families and individuals at high risk of developing gastric cancer, including criteria for referral, classification, diagnostics, and periodic gastric surveillance. These guidelines are not limited to HDGC and are therefore partially complementary to the guidelines on hereditary diffuse gastric cancer of the international gastric cancer linkage consortium (IGCLC 2010). In order to optimize the care and increase the knowledge on hereditary gastric cancer it is important to centralize medical care for these patients. National and international collaboration is warranted to improve the quality of research by increasing the size of study cohorts.

Keywords

Familial gastric cancer Hereditary diffuse gastric cancer CDH1 Gastric surveillance Prophylactic gastrectomy Intestinal gastric cancer 

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Copyright information

© Springer Science+Business Media B.V. 2012

Authors and Affiliations

  • Irma Kluijt
    • 1
  • Rolf H. Sijmons
    • 2
  • Nicoline Hoogerbrugge
    • 3
  • John T. Plukker
    • 4
  • Daphne de Jong
    • 5
  • J. Han van Krieken
    • 6
  • Richard van Hillegersberg
    • 7
  • Marjolijn Ligtenberg
    • 3
    • 6
  • Eveline Bleiker
    • 8
  • Anemieke Cats
    • 9
  1. 1.Family Cancer ClinicThe Netherlands Cancer InstituteAmsterdamThe Netherlands
  2. 2.Departments of Genetics, University Medical Centre GroningenUniversity of GroningenGroningenThe Netherlands
  3. 3.Departments of Human Genetics, Medical CentreRadboud University NijmegenNijmegenThe Netherlands
  4. 4.Surgical Oncology, University Medical Centre GroningenUniversity of GroningenGroningenThe Netherlands
  5. 5.Departments of PathologyThe Netherlands Cancer InstituteAmsterdamThe Netherlands
  6. 6.Pathology, Medical CentreRadboud University NijmegenNijmegenThe Netherlands
  7. 7.Department of Surgical OncologyUniversity Medical Centre UtrechtUtrechtThe Netherlands
  8. 8.Psychosocial Research and EpidemiologyThe Netherlands Cancer InstituteAmsterdamThe Netherlands
  9. 9.GastroenterologyThe Netherlands Cancer InstituteAmsterdamThe Netherlands

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