Universität zu Köln

Engel, Christoph ORCID: 0000-0002-7247-282X, Loeffler, Markus, Steinke, Verena, Rahner, Nils, Holinski-Feder, Elke, Dietmaier, Wolfgang, Schackert, Hans K., Goergens, Heike, Doeberitz, Magnus von Knebel, Goecke, Timm O., Schmiegel, Wolff, Buettner, Reinhard, Moeslein, Gabriela, Letteboer, Tom G. W., Garcia, Encarna Gomez, Hes, Frederik J., Hoogerbrugge, Nicoline ORCID: 0000-0003-2393-8141, Menko, Fred H., van Os, Theo A. M., Sijmons, Rolf H., Wagner, Anja, Kluijt, Irma, Propping, Peter and Vasen, Hans F. A. (2012). Risks of Less Common Cancers in Proven Mutation Carriers With Lynch Syndrome. J. Clin. Oncol., 30 (35). S. 4409 - 4416. ALEXANDRIA: AMER SOC CLINICAL ONCOLOGY. ISSN 0732-183X

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Abstract

Purpose Patients with Lynch syndrome are at high risk for colon and endometrial cancer, but also at an elevated risk for other less common cancers. The purpose of this retrospective cohort study was to provide risk estimates for these less common cancers in proven carriers of pathogenic mutations in the mismatch repair (MMR) genes MLH1, MSH2, and MSH6. Patients and Methods Data were pooled from the German and Dutch national Lynch syndrome registries. Seven different cancer types were analyzed: stomach, small bowel, urinary bladder, other urothelial, breast, ovarian, and prostate cancer. Age-, sex-and MMR gene-specific cumulative risks (CRs) were calculated using the Kaplan-Meier method. Sex-specific incidence rates were compared with general population incidence rates by calculating standardized incidence ratios (SIRs). Multivariate Cox regression analysis was used to estimate the impact of sex and mutated gene on cancer risk. Results The cohort comprised 2,118 MMR gene mutation carriers (MLH1, n = 806; MSH2, n = 1,004; MSH6, n = 308). All cancers were significantly more frequent than in the general population. The highest risks were found for male small bowel cancer (SIR, 251; 95% CI, 177 to 346; CR at 70 years, 12.0; 95% CI, 5.7 to 18.2). Breast cancer showed an SIR of 1.9 (95% CI, 1.4 to 2.4) and a CR of 14.4 (95% CI, 9.5 to 19.3). MSH2 mutation carriers had a considerably higher risk of developing urothelial cancer than MLH1 or MSH6 carriers. Conclusion The sex-and gene-specific differences of less common cancer risks should be taken into account in cancer surveillance and prevention programs for patients with Lynch syndrome. J Clin Oncol 30: 4409-4415. (C) 2012 by American Society of Clinical Oncology

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Engel, Christoph UNSPECIFIED orcid.org/0000-0002-7247-282X UNSPECIFIED Loeffler, Markus UNSPECIFIED UNSPECIFIED UNSPECIFIED Steinke, Verena UNSPECIFIED UNSPECIFIED UNSPECIFIED Rahner, Nils UNSPECIFIED UNSPECIFIED UNSPECIFIED Holinski-Feder, Elke UNSPECIFIED UNSPECIFIED UNSPECIFIED Dietmaier, Wolfgang UNSPECIFIED UNSPECIFIED UNSPECIFIED Schackert, Hans K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Goergens, Heike UNSPECIFIED UNSPECIFIED UNSPECIFIED Doeberitz, Magnus von Knebel UNSPECIFIED UNSPECIFIED UNSPECIFIED Goecke, Timm O. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmiegel, Wolff UNSPECIFIED UNSPECIFIED UNSPECIFIED Buettner, Reinhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Moeslein, Gabriela UNSPECIFIED UNSPECIFIED UNSPECIFIED Letteboer, Tom G. W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Garcia, Encarna Gomez UNSPECIFIED UNSPECIFIED UNSPECIFIED Hes, Frederik J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hoogerbrugge, Nicoline UNSPECIFIED orcid.org/0000-0003-2393-8141 UNSPECIFIED Menko, Fred H. UNSPECIFIED UNSPECIFIED UNSPECIFIED van Os, Theo A. M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sijmons, Rolf H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wagner, Anja UNSPECIFIED UNSPECIFIED UNSPECIFIED Kluijt, Irma UNSPECIFIED UNSPECIFIED UNSPECIFIED Propping, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Vasen, Hans F. A. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-476815
DOI:	10.1200/JCO.2012.43.2278
Journal or Publication Title:	J. Clin. Oncol.
Volume:	30
Number:	35
Page Range:	S. 4409 - 4416
Date:	2012
Publisher:	AMER SOC CLINICAL ONCOLOGY
Place of Publication:	ALEXANDRIA
ISSN:	0732-183X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language NONPOLYPOSIS COLORECTAL-CANCER; MISMATCH-REPAIR GENES; BETHESDA GUIDELINES; FAMILIES; MSH2; MLH1; HNPCC; NETHERLANDS; INCREASES; ADENOMAS Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/47681