Universität zu Köln

Bucksch, Karolin, Zachariae, Silke, Aretz, Stefan, Buettner, Reinhard, Holinski-Feder, Elke, Holzapfel, Stefanie, Hueneburg, Robert, Kloor, Matthias, Doeberitz, Magnus von Knebel, Morak, Monika, Moeslein, Gabriela, Nattermann, Jacob, Perne, Claudia, Rahner, Nils, Schmiegel, Wolff, Schulmann, Karsten, Steinke-Lange, Verena, Strassburg, Christian P., Vangala, Deepak B., Weitz, Juergen, Loeffler, Markus and Engel, Christoph ORCID: 0000-0002-7247-282X (2020). Cancer risks in Lynch syndrome, Lynch-like syndrome, and familial colorectal cancer type X: a prospective cohort study. BMC Cancer, 20 (1). LONDON: BMC. ISSN 1471-2407

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Abstract

BackgroundIndividuals with pathogenic germline variants in DNA mismatch repair (MMR) genes are at increased risk of developing colorectal, endometrial and other cancers (Lynch syndrome, LS). While previous studies have extensively described cancer risks in LS, cancer risks in individuals from families without detectable MMR gene defects despite MMR deficiency (Lynch-like syndrome, LLS), and in individuals from families fulfilling the Amsterdam-II criteria without any signs of MMR deficiency (familial colorectal cancer type X, FCCX) are less well studied. The aim of this prospective study was to characterise the risk for different cancer types in LS, LLS, and FCCX, and to compare these with the cancer risks in the general population.MethodsData was taken from the registry of the German Consortium for Familial Intestinal Cancer, where individuals were followed up prospectively within the framework of an intensified surveillance programme at recommended annual examination intervals. A total of 1120 LS, 594 LLS, and 116 FCCX individuals were analysed. From this total sample, eight different cohorts were defined, in which age-dependent cumulative risks and standardised incidence ratios were calculated regarding the first incident occurrence of any, colorectal, stomach, small bowel, urothelial, female breast, ovarian, and endometrial cancer, separately for LS, LLS, and FCCX.ResultsThe number of individuals at risk for first incident cancer ranged from 322 to 1102 in LS, 120 to 586 in LLS, and 40 to 116 in FCCX, depending on the cancer type of interest. For most cancer types, higher risks were observed in LS compared to LLS, FCCX, and the general population. Risks for any, colorectal, stomach, urothelial, and endometrial cancer were significantly higher in LLS compared to the general population. No significantly increased risks could be detected in FCCX compared to LLS patients, and the general population. Colorectal and endometrial cancer risks tended to be higher in LLS than in FCCX.ConclusionsThe characterisation of cancer risks in patients with LLS and FCCX is important to develop appropriate surveillance programmes for these specific intermediate risk groups. Larger prospective studies are needed to obtain more precise risk estimates.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Bucksch, Karolin UNSPECIFIED UNSPECIFIED UNSPECIFIED Zachariae, Silke UNSPECIFIED UNSPECIFIED UNSPECIFIED Aretz, Stefan UNSPECIFIED UNSPECIFIED UNSPECIFIED Buettner, Reinhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Holinski-Feder, Elke UNSPECIFIED UNSPECIFIED UNSPECIFIED Holzapfel, Stefanie UNSPECIFIED UNSPECIFIED UNSPECIFIED Hueneburg, Robert UNSPECIFIED UNSPECIFIED UNSPECIFIED Kloor, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED Doeberitz, Magnus von Knebel UNSPECIFIED UNSPECIFIED UNSPECIFIED Morak, Monika UNSPECIFIED UNSPECIFIED UNSPECIFIED Moeslein, Gabriela UNSPECIFIED UNSPECIFIED UNSPECIFIED Nattermann, Jacob UNSPECIFIED UNSPECIFIED UNSPECIFIED Perne, Claudia UNSPECIFIED UNSPECIFIED UNSPECIFIED Rahner, Nils UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmiegel, Wolff UNSPECIFIED UNSPECIFIED UNSPECIFIED Schulmann, Karsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Steinke-Lange, Verena UNSPECIFIED UNSPECIFIED UNSPECIFIED Strassburg, Christian P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Vangala, Deepak B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weitz, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Loeffler, Markus UNSPECIFIED UNSPECIFIED UNSPECIFIED Engel, Christoph UNSPECIFIED orcid.org/0000-0002-7247-282X UNSPECIFIED
URN:	urn:nbn:de:hbz:38-333038
DOI:	10.1186/s12885-020-06926-x
Journal or Publication Title:	BMC Cancer
Volume:	20
Number:	1
Date:	2020
Publisher:	BMC
Place of Publication:	LONDON
ISSN:	1471-2407
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MUTATION CARRIERS; CLINICAL-CRITERIA; GENE-MUTATIONS; MSH2; MLH1 Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/33303