Journal of Clinical Medicine (Jul 2020)
Risk-Reducing Gynecological Surgery in Lynch Syndrome: Results of an International Survey from the Prospective Lynch Syndrome Database
- Mev Dominguez-Valentin,
- Toni T. Seppälä,
- Christoph Engel,
- Stefan Aretz,
- Finlay Macrae,
- Ingrid Winship,
- Gabriel Capella,
- Huw Thomas,
- Eivind Hovig,
- Maartje Nielsen,
- Rolf H Sijmons,
- Lucio Bertario,
- Bernardo Bonanni,
- Maria Grazia Tibiletti,
- Giulia Martina Cavestro,
- Miriam Mints,
- Nathan Gluck,
- Lior Katz,
- Karl Heinimann,
- Carlos A. Vaccaro,
- Kate Green,
- Fiona Lalloo,
- James Hill,
- Wolff Schmiegel,
- Deepak Vangala,
- Claudia Perne,
- Hans-Georg Strauß,
- Johanna Tecklenburg,
- Elke Holinski-Feder,
- Verena Steinke-Lange,
- Jukka-Pekka Mecklin,
- John-Paul Plazzer,
- Marta Pineda,
- Matilde Navarro,
- Joan Brunet Vidal,
- Revital Kariv,
- Guy Rosner,
- Tamara Alejandra Piñero,
- María Laura Gonzalez,
- Pablo Kalfayan,
- Julian R. Sampson,
- Neil A. J. Ryan,
- D. Gareth Evans,
- Pål Møller,
- Emma J. Crosbie
Affiliations
- Mev Dominguez-Valentin
- Department of Tumor Biology, Institute of Cancer Research, The Norwegian Radium Hospital, Part of Oslo University Hospital, 0379 Oslo, Norway
- Toni T. Seppälä
- Department of Abdominal Surgery, Helsinki University Central Hospital, 00029 Helsinki, Finland
- Christoph Engel
- Institute for Medical Informatics, Statistics and Epidemiology, University of Leipzig, 04107 Leipzig, Germany
- Stefan Aretz
- Institute of Human Genetics, University of Bonn, 53127 Bonn, Germany
- Finlay Macrae
- Colorectal Medicine and Genetics, The Royal Melbourne Hospital, 3052 Melbourne, Australia
- Ingrid Winship
- Colorectal Medicine and Genetics, The Royal Melbourne Hospital, 3052 Melbourne, Australia
- Gabriel Capella
- Hereditary Cancer Program, Catalan Institute of Oncology, Insititut d’Investigació Biomèdica de Bellvitge (IDIBELL), ONCOBELL Program, L’Hospitalet de Llobregat, 08908 Barcelona, Spain
- Huw Thomas
- Department of Surgery and Cancer, St Mark’s Hospital, Imperial College London, London HA1 3UJ, UK
- Eivind Hovig
- Department of Tumor Biology, Institute of Cancer Research, The Norwegian Radium Hospital, Part of Oslo University Hospital, 0379 Oslo, Norway
- Maartje Nielsen
- Leids Universitair Medisch Centrum, Department of Clinical Genetics, 2300RC Leiden, The Netherlands
- Rolf H Sijmons
- Department of Genetics, University of Groningen, University Medical Center Groningen, 9713GZ Groningen, The Netherlands
- Lucio Bertario
- Scientific Consultant of the Division of Prevention and Genetic Oncology, European Institute of Oncology, Fondazione IRCCS Isrtituto nazionale dei Tumori, 20141 Milan, Italy
- Bernardo Bonanni
- Division of Prevention and Genetic Oncology, European Institute of Oncology, 20141 Milan, Italy
- Maria Grazia Tibiletti
- Ospedale di Circolo ASST Settelaghi, Centro di Ricerca Tumori Eredo-Familiari, Università dell’Insubria, 21100 Varese, Italy
- Giulia Martina Cavestro
- Gastroenterology and Gastrointestinal Endoscopy Unit, Vita-Salute San Raffaele University, San Raffaele Scientific Institute, 20132 Milan, Italy
- Miriam Mints
- Karolinska Institutet, 171 76 Stockholm, Sweden
- Nathan Gluck
- Department of Gastroenterology, Tel-Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel-Aviv University, 64239 Tel Aviv, Israel
- Lior Katz
- Department of Gastroenterology and Hepatology, Hadassah Medical Center, 91120 Jerusalem, Israel
- Karl Heinimann
- Medical Genetics, Institute for Medical Genetics and Pathology, University Hospital Basel, 4031 Basel, Switzerland
- Carlos A. Vaccaro
- Hereditary Cancer Program (PROCANHE) Hospital Italiano de Buenos Aires, C1199ABB Ciudad Autónoma de Buenos Aires, Argentina
- Kate Green
- University of Manchester & Manchester University NHS Foundation Trust, Manchester M13 9WL, UK
- Fiona Lalloo
- University of Manchester & Manchester University NHS Foundation Trust, Manchester M13 9WL, UK
- James Hill
- Department of Surgery, Manchester University NHS Foundation Trust and University of Manchester, Manchester M13 9WL, UK
- Wolff Schmiegel
- Department of Medicine, Knappschaftskrankenhaus, Ruhr-University Bochum, 44801 Bochum, Germany
- Deepak Vangala
- Department of Medicine, Knappschaftskrankenhaus, Ruhr-University Bochum, 44892 Bochum, Germany
- Claudia Perne
- Institute of Human Genetics, University of Bonn, 53127 Bonn, Germany
- Hans-Georg Strauß
- Department of Gynecology, University Clinics, Martin-Luther University, D-06097 Halle (Saale), Germany
- Johanna Tecklenburg
- Institute of Human Genetics, Hannover Medical School, 30625 Hannover, Germany
- Elke Holinski-Feder
- Medizinische Klinik und Poliklinik IV, Campus Innenstadt, Klinikum der Universität München, 80336 Munich, Germany
- Verena Steinke-Lange
- Medizinische Klinik und Poliklinik IV, Campus Innenstadt, Klinikum der Universität München, 80336 Munich, Germany
- Jukka-Pekka Mecklin
- Faculty of Sport and Health Sciences, University of Jyväskylä, 40014 Jyväskylä, Finland
- John-Paul Plazzer
- Colorectal Medicine and Genetics, The Royal Melbourne Hospital, 3052 Melbourne, Australia
- Marta Pineda
- Hereditary Cancer Program, Catalan Institute of Oncology, Insititut d’Investigació Biomèdica de Bellvitge (IDIBELL), ONCOBELL Program, L’Hospitalet de Llobregat, 08908 Barcelona, Spain
- Matilde Navarro
- Hereditary Cancer Program, Catalan Institute of Oncology, Insititut d’Investigació Biomèdica de Bellvitge (IDIBELL), ONCOBELL Program, L’Hospitalet de Llobregat, 08908 Barcelona, Spain
- Joan Brunet Vidal
- Hereditary Cancer Program, Catalan Institute of Oncology, Insititut d’Investigació Biomèdica de Bellvitge (IDIBELL), ONCOBELL Program, L’Hospitalet de Llobregat, 08908 Barcelona, Spain
- Revital Kariv
- Department of Gastroenterology, Tel-Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel-Aviv University, 64239 Tel Aviv, Israel
- Guy Rosner
- Department of Gastroenterology, Tel-Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel-Aviv University, 64239 Tel Aviv, Israel
- Tamara Alejandra Piñero
- Hereditary Cancer Program (PROCANHE) Hospital Italiano de Buenos Aires, C1199ABB Ciudad Autónoma de Buenos Aires, Argentina
- María Laura Gonzalez
- Hereditary Cancer Program (PROCANHE) Hospital Italiano de Buenos Aires, C1199ABB Ciudad Autónoma de Buenos Aires, Argentina
- Pablo Kalfayan
- Hereditary Cancer Program (PROCANHE) Hospital Italiano de Buenos Aires, C1199ABB Ciudad Autónoma de Buenos Aires, Argentina
- Julian R. Sampson
- Institute of Medical Genetics, Division of Cancer and Genetics, Cardiff University School of Medicine, Cardiff CF14 4XN, UK
- Neil A. J. Ryan
- University of Manchester & Manchester University NHS Foundation Trust, Manchester M13 9WL, UK
- D. Gareth Evans
- University of Manchester & Manchester University NHS Foundation Trust, Manchester M13 9WL, UK
- Pål Møller
- Department of Tumor Biology, Institute of Cancer Research, The Norwegian Radium Hospital, Part of Oslo University Hospital, 0379 Oslo, Norway
- Emma J. Crosbie
- University of Manchester & Manchester University NHS Foundation Trust, Manchester M13 9WL, UK
- DOI
- https://doi.org/10.3390/jcm9072290
- Journal volume & issue
-
Vol. 9,
no. 7
p. 2290
Abstract
Purpose: To survey risk-reducing hysterectomy and bilateral salpingo-oophorectomy (BSO) practice and advice regarding hormone replacement therapy (HRT) in women with Lynch syndrome. Methods: We conducted a survey in 31 contributing centers from the Prospective Lynch Syndrome Database (PLSD), which incorporates 18 countries worldwide. The survey covered local policies for risk-reducing hysterectomy and BSO in Lynch syndrome, the timing when these measures are offered, the involvement of stakeholders and advice regarding HRT. Results: Risk-reducing hysterectomy and BSO are offered to path_MLH1 and path_MSH2 carriers in 20/21 (95%) contributing centers, to path_MSH6 carriers in 19/21 (91%) and to path_PMS2 carriers in 14/21 (67%). Regarding the involvement of stakeholders, there is global agreement (~90%) that risk-reducing surgery should be offered to women, and that this discussion may involve gynecologists, genetic counselors and/or medical geneticists. Prescription of estrogen-only HRT is offered by 15/21 (71%) centers to women of variable age range (35–55 years). Conclusions: Most centers offer risk-reducing gynecological surgery to carriers of path_MLH1, path_MSH2 and path_MSH6 variants but less so for path_PMS2 carriers. There is wide variation in how, when and to whom this is offered. The Manchester International Consensus Group developed recommendations to harmonize clinical practice across centers, but there is a clear need for more research.
Keywords
