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a Department of
Oncology, Cambridge Institute for Medical Research and Strangeways
Research Laboratories, University of Cambridge, Addenbrooke's
Hospital, Cambridge CB2 2XY, UK, b IPATIMUP and Faculty of Medicine,
University of Porto, 4200 Porto, Portugal, c Department of
Preventive Medicine and Public Health, Creighton University, Omaha, NE
68178, USA, d National
Cancer Center Research Institute, Chuo-ku, Tokyo 104-0045, Japan, e Department of Genetics and the
Center for Human Genetics, Case Western Reserve University and
University Hospitals of Cleveland, Cleveland, OH 44106-6055, USA, f Division of
Gastroenterology, University of Virginia, Charlottesville, VA
22906-0013, USA, g Departments
of Surgery and Medical Genetics, Henry Ford Hospital, Detroit, MI
48202, USA, h
Department of Pathology and Laboratory
Medicine, The University of British Columbia, Vancouver V6H 3V4,
Canada, i Departments
of Medicine and Medical Genetics, University of Birmingham, Birmingham
B15 2TG,
UK, j Section
of Genetics, Victoria General Hospital, Victoria, BC V8Z 6R5,
Canada, k Departments
of Pathology and Surgery, Technical University of Munich, D-81675
Munich,
Germany, l Department
of Surgery, Aberdeen Royal Infirmary, Aberdeen AB9 2ZB,
UK, m Department
of Pathology, Addenbrooke's Hospital, Cambridge CB2 2QQ,
UK, n Istituto
Policattedra di Scienze Chirurgiche, Universita degli Studi di Siena,
53100 Siena,
Italy
Correspondence to: Dr Caldas
Families with autosomal dominant inherited predisposition to
gastric cancer have been described. More recently, germline
E-cadherin/CDH1 mutations have been identified in hereditary diffuse gastric cancer kindred. The need to have protocols to manage and counsel these families in the clinic led a group of geneticists, gastroenterologists, surgeons, oncologists, pathologists, and molecular biologists to
convene a workshop to produce consensus statements and guidelines for
familial gastric cancer. Review of the available cancer pathology from
people belonging to families with documented germline
E-cadherin/CDH1 mutations confirmed that the gastric cancers were all of the diffuse type. Criteria to define the different types of familial gastric cancer
syndromes were agreed. Foremost among these criteria was that review of
histopathology should be part of the evaluation of any family with
aggregation of gastric cancer cases. Guidelines for genetic testing and
counselling in hereditary diffuse gastric cancer were produced.
Finally, a proposed strategy for clinical management in families with
high penetrance autosomal dominant predisposition to gastric cancer was defined.
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