Hueneburg, Robert, Aretz, Stefan ORCID: 0000-0002-5228-1890, Buettner, Reinhard, Daum, Severin, Engel, Christoph ORCID: 0000-0002-7247-282X, Fechner, Guido, Habermann, Jens K., Heling, Dominik, Hoffmann, Katrin, Holinski-Feder, Elke, Kloor, Matthias, von Knebel-Doeberitz, Magnus, Loeffler, Markus, Moeslein, Gabriela, Perne, Claudia, Redler, Silke, Riess, Olaf, Schmiegel, Wolff, Seufferlein, Thomas, Siebers-Renelt, Ulrike, Steinke-Lange, Verena, Tecklenburg, Johanna, Vangala, Deepak, Vilz, Tim, Weitz, Juergen, Wiedenmann, Bertram, Strassburg, Christian P. and Nattermann, Jacob (2019). Current recommendations for surveillance, risk reduction and therapy in Lynch syndrome patients. Z. Gastroent., 57 (11). S. 1309 - 1321. STUTTGART: GEORG THIEME VERLAG KG. ISSN 1439-7803

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Abstract

Introduction Lynch syndrome (LS) is the most common hereditary colorectal cancer syndrome and accounts for similar to 3 % of all CRCs. This autosomal dominant disorder is caused by germline mutations in DNA mismatch repair genes (MLH1, MSH2, MSH6, PMS2, and EPCAM). One in 300 individuals of the general population are considered to be mutation carriers (300 000 individuals/Germany). Mutation carriers are at a high CRC risk of 15-46 % till the age of 75 years. LS also includes a variety of extracolonic malignancies such as endometrial, small bowel, gastric, urothelial, and other cancers. Methods The German Consortium for Familial Intestinal Cancer consists of 14 university centers in Germany. The aim of the consortium is to develop and evaluate surveillance programs and to further translate the results in clinical care. We have revisited and updated the clinical management guidelines for LS patients in Germany. Results A surveillance colonoscopy should be performed every 12-24 months starting at the age of 25 years. At diagnosis of first colorectal cancer, an oncological resection is advised, an extended resection (colectomy with ileorectal anastomosis) has to be discussed with the patient. The lifetime risk for gastric cancer is 0.2-13 %. Gastric cancers detected during surveillance have a lower tumor stage compared to symptom-driven detection. The lifetime risk for small bowel cancer is 4-8 %. About half of small bowel cancer is located in the duodenum and occurs before the age of 35 years in 10 % of all cases. Accordingly, patients are advised to undergo an esophagogastroduodenoscopy every 12-36 months starting by the age of 25 years. Conclusion LS colonic and extracolonic clinical management, surveillance and therapy are complex and several aspects remain unclear. In the future, surveillance and clinical management need to be more tailored to gene and gender. Future prospective trials are needed.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Hueneburg, RobertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aretz, StefanUNSPECIFIEDorcid.org/0000-0002-5228-1890UNSPECIFIED
Buettner, ReinhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Daum, SeverinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Engel, ChristophUNSPECIFIEDorcid.org/0000-0002-7247-282XUNSPECIFIED
Fechner, GuidoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Habermann, Jens K.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heling, DominikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoffmann, KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Holinski-Feder, ElkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kloor, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Knebel-Doeberitz, MagnusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Loeffler, MarkusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Moeslein, GabrielaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perne, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Redler, SilkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Riess, OlafUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schmiegel, WolffUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Seufferlein, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Siebers-Renelt, UlrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Steinke-Lange, VerenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tecklenburg, JohannaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vangala, DeepakUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vilz, TimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weitz, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wiedenmann, BertramUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Strassburg, Christian P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nattermann, JacobUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-129200
DOI: 10.1055/a-1008-9827
Journal or Publication Title: Z. Gastroent.
Volume: 57
Number: 11
Page Range: S. 1309 - 1321
Date: 2019
Publisher: GEORG THIEME VERLAG KG
Place of Publication: STUTTGART
ISSN: 1439-7803
Language: German
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
NONPOLYPOSIS COLORECTAL-CANCER; SMALL-BOWEL CANCER; COLONOSCOPIC SURVEILLANCE; GASTRIC-CANCER; ENDOSCOPIC SURVEILLANCE; ENDOMETRIAL CANCER; MUTATION CARRIERS; URINE CYTOLOGY; LONG-TERM; ADENOMASMultiple languages
Gastroenterology & HepatologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/12920

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