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Guidelines for the clinical management of Lynch syndrome (HNPCC)
  1. Hans F.A. Vasen (hfavasen{at}stoet.nl)
  1. Department of Gastroenterology, Leiden University Medical Centre, Netherlands
    1. Gabriele Möslein
    1. Department of Surgery, St.Josefs Hospital Bochum-Linden (Helios), Bochum, Germany
      1. Angel Alonso
      1. Department of Medical Genetics, Hospital Virgen del Camino, Pamplona, Spain
        1. Inge Bernstein
        1. Department of Gastroenterology, Hvidrove Hospital, Hvidrove, Denmark
          1. Lucio Bertario
          1. Department of Surgery, Hospital Tumori, Milan, Italy
            1. Ignacio Blanco
            1. Department of Genetic Counselling, Prevention and Cancer, Catelonian Inst. of Oncology, Barcelona, Spain
              1. John Burn
              1. Institute of Human Genetics, New Castle upon Tyne, United Kingdom
                1. Gabriel Capella
                1. Institute Catala D'Oncologia, Barcelona, Spain
                  1. Christoph Engel
                  1. Institute of Medical Informatics, Statistics and Epidemiology, University of Leipzig, Germany
                    1. Ian Frayling
                    1. Institute of Medical Genetics, Cardiff, United Kingdom
                      1. Waltraut Friedl
                      1. Institute of Human Genetics, University Clinics, Bonn, Germany
                        1. Frederik J. Hes
                        1. Department of Clinical Genetics, Leiden University Medical Centre, Netherlands
                          1. Shirley Hodgson
                          1. Department of Clinical Genetics, St. George's Hospital, London, United Kingdom
                            1. Jukka-Pekka Mecklin
                            1. Department of Surgery, Jyvaskyla Central Hospital, Jyvaskyla, Finland
                              1. Pål Møller
                              1. Department of Genetics, Norwegian Radium Hospital, Oslo, Norway
                                1. Fokko N. Nagengast
                                1. Department of Gastroenterology, University Medical Centre, Radboud, Nijmegen, Netherlands
                                  1. Yann Parc
                                  1. Department of Digestive Surgery, Hospital Saint-Antoine, University Pierre et Marie, Paris, France
                                    1. Laura Renkonen-Sinisalo
                                    1. Department of Surgery, Helsinki University Central Hospital, Helsinki, Finland
                                      1. Julian R. Sampson
                                      1. Institute of Medical Genetics, Cardiff, United Kingdom
                                        1. Astrid Stormorken
                                        1. Department of Medical Genetics, Ullevål University Hospital, Oslo, Norway
                                          1. Juul Wijnen
                                          1. Department of Clinical Genetics, Leiden University Medical Centre, Netherlands

                                            Abstract

                                            The Lynch syndrome (HNPCC) is characterized by the development of colorectal cancer, endometrial cancer and various other cancers and is caused by a mutation in one of the mismatch repair (MMR) genes: MLH1, MSH2, MSH6 or PMS2.The discovery of these genes 15 years ago, has led to the identification of large numbers of affected families. In April 2006, a workshop was organized by a group of European experts in Hereditary Gastrointestinal Cancer (the Mallorca-group) aiming to establish guidelines for the clinical management of Lynch syndrome. Twenty-one experts from nine European countries participated in this workshop. Prior to the meeting, various participants prepared the key management issues of debate according to the latest publications. A systematic literature search using Pubmed and the Cochrane Database of Systematic Reviews, reference lists of retrieved articles, and manual searches of relevant articles was performed. During the workshop all recommendations were discussed in detail. Because most of the studies that form the basis for the recommendations were descriptive and/or retrospective of nature, many of them were based on expert opinion. The guidelines described in this manuscript may be helpful to the appropriate management of Lynch syndrome families. In order to further improve the care of these families, prospective controlled studies should be undertaken.

                                            • Lynch syndrome
                                            • MSI
                                            • guidelines
                                            • immunohistochemistry
                                            • surveillance

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