title: Hereditary cancer registries improve the care of patients with a genetic predisposition to cancer: contributions from the Dutch Lynch syndrome registry.
abstract: The Dutch Hereditary Cancer Registry was established in 1985 with the support of the Ministry of Health (VWS). The aims of the registry are: (1) to promote the identification of families with hereditary cancer, (2) to encourage the participation in surveillance programs of individuals at high risk, (3) to ensure the continuity of lifelong surveillance examinations, and (4) to promote research, in particular the improvement of surveillance protocols. During its early days the registry provided assistance with family investigations and the collection of medical data, and recommended surveillance when a family fulfilled specific diagnostic criteria. Since 2000 the registry has focused on family follow-up, and ensuring the quality of surveillance programs and appropriate clinical management. Since its founding, the registry has identified over 10,000 high-risk individuals with a diverse array of hereditary cancer syndromes. All were encouraged to participate in prevention programmes. The registry has published a number of studies that evaluated the outcome of surveillance protocols for colorectal cancer (CRC) in Lynch syndrome, as well as in familial colorectal cancer. In 2006, evaluation of the effect of registration and colonoscopic surveillance on the mortality rate associated with colorectal cancer (CRC) showed that the policy led to a substantial decrease in the mortality rate associated with CRC. Following discovery of MMR gene defects, the first predictive model that could select families for genetic testing was published by the Leiden group. In addition, over the years the registry has produced many cancer risk studies that have helped to develop appropriate surveillance protocols. Hereditary cancer registries in general, and the Lynch syndrome registry in particular, play an important role in improving the clinical management of affected families.
authors.affiliation: Department of Gastroenterology and Hepatology, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands. hfavasen@stoet.nl.|Hereditary Cancer Registry, Leiden, The Netherlands. hfavasen@stoet.nl.
authors.forename: Hans F A
authors.initials: HF
authors.lastname: Vasen
authors.affiliation: Department of Clinical Genetics, University Medical Centre, Utrecht, The Netherlands.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Velthuizen
authors.affiliation: Department of Gastroenterology and Hepatology, University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: Jan H
authors.initials: JH
authors.lastname: Kleibeuker
authors.affiliation: Cancer Family Clinic, Netherlands Cancer Institute, Amsterdam, The Netherlands.
authors.forename: Fred H
authors.initials: FH
authors.lastname: Menko
authors.affiliation: Department of Gastroenterology and Hepatology, Slingerland Ziekenhuis, Doetinchem, The Netherlands.
authors.forename: Fokke M
authors.initials: FM
authors.lastname: Nagengast
authors.affiliation: Department of Gastroenterology and Hepatology, Netherlands Cancer Institute, Amsterdam, The Netherlands.
authors.forename: Annemieke
authors.initials: A
authors.lastname: Cats
authors.affiliation: Department of Gastroenterology and Hepatology, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: Andrea E
authors.initials: AE
authors.lastname: van der Meulen-de Jong
authors.affiliation: Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: Martijn H
authors.initials: MH
authors.lastname: Breuning
authors.affiliation: Department of Surgery, Elizabeth Hospital, Tilburg, The Netherlands.
authors.forename: Anne J
authors.initials: AJ
authors.lastname: Roukema
authors.affiliation: Hereditary Cancer Registry, Leiden, The Netherlands.
authors.forename: Inge
authors.initials: I
authors.lastname: van Leeuwen-Cornelisse
authors.affiliation: Department of Gastroenterology and Hepatology, Isala Clinics, Zwolle, The Netherlands.
authors.forename: Wouter H
authors.initials: WH
authors.lastname: de Vos Tot Nederveen Cappel
authors.affiliation: Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: Juul T
authors.initials: JT
authors.lastname: Wijnen
mesh_terms: D003113:Colonoscopy; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D053843:DNA Mismatch Repair / Q000235:genetics; D045643:DNA Repair Enzymes / Q000235:genetics; D055088:Early Detection of Cancer / Q000379:methods*; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing / Q000379:methods*; D006801:Humans; D015994:Incidence; D015201:Meta-Analysis as Topic; D009154:Mutation; D009426:Netherlands / Q000453:epidemiology; D012042:Registries
chemical_list: D045643:DNA Repair Enzymes
keywords: Cancer risk; Follow-up system; Hereditary cancer; Identification; Lynch syndrome; Registry; Surveillance