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Towards gene-and gender-based risk estimates in Lynch syndrome; Age-specific incidences for 13 extra-colorectal cancer types

Therkildsen, Christina LU ; Ladelund, Steen; Smith-Hansen, Lars; Lindberg, Lars Joachim and Nilbert, Mef LU (2017) In British Journal of Cancer 117(11). p.1702-1710
Abstract

Background:In Lynch syndrome, inherited mismatch repair (MMR) defects predispose to colorectal cancer and to a wide spectrum of extra-colorectal tumours. Utilising a cohort study design, we aimed to determine the risk of extra-colorectal cancer and to identify yet unrecognised tumour types.Methods:Data from 1624 Lynch syndrome mutation carriers in the Danish hereditary non-polyposis colorectal cancer register were used to estimate the sex-and age-specific incidence rate ratios (IRRs) for 30 extra-colorectal malignancies with comparison to the general population.Results:Significantly increased IRRs were identified for 13 cancer types with differences related to gender, age and disease-predisposing gene. The different cancer types showed... (More)

Background:In Lynch syndrome, inherited mismatch repair (MMR) defects predispose to colorectal cancer and to a wide spectrum of extra-colorectal tumours. Utilising a cohort study design, we aimed to determine the risk of extra-colorectal cancer and to identify yet unrecognised tumour types.Methods:Data from 1624 Lynch syndrome mutation carriers in the Danish hereditary non-polyposis colorectal cancer register were used to estimate the sex-and age-specific incidence rate ratios (IRRs) for 30 extra-colorectal malignancies with comparison to the general population.Results:Significantly increased IRRs were identified for 13 cancer types with differences related to gender, age and disease-predisposing gene. The different cancer types showed variable peak age incidence rates (IRs) with the highest IRs for ovarian cancer at age 30-49 years, for endometrial cancer, breast cancer, renal cell cancer and brain tumours at age 50-69 years, and for urothelial cancer, small bowel cancer, gastric cancer, pancreatic cancer and skin tumours after age 70.Conclusions:The broad spectrum of tumour types that develop at an increased incidence defines Lynch syndrome as a multi-tumour syndrome. The variable incidences in relation to age, gender and gene suggest a need for individualised surveillance.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
breast cancer, colorectal cancer, hereditary non-polyposis colorectal cancer, mismatch repair genes, pancreatic cancer, prostate cancer
in
British Journal of Cancer
volume
117
issue
11
pages
9 pages
publisher
Nature Publishing Group
external identifiers
  • scopus:85034841799
  • wos:000416511700015
ISSN
0007-0920
DOI
10.1038/bjc.2017.348
language
English
LU publication?
yes
id
f2168a9a-2dfa-4e63-aa7d-37bae7f07996
date added to LUP
2017-12-14 12:46:48
date last changed
2018-01-16 13:27:50
@article{f2168a9a-2dfa-4e63-aa7d-37bae7f07996,
  abstract     = {<p>Background:In Lynch syndrome, inherited mismatch repair (MMR) defects predispose to colorectal cancer and to a wide spectrum of extra-colorectal tumours. Utilising a cohort study design, we aimed to determine the risk of extra-colorectal cancer and to identify yet unrecognised tumour types.Methods:Data from 1624 Lynch syndrome mutation carriers in the Danish hereditary non-polyposis colorectal cancer register were used to estimate the sex-and age-specific incidence rate ratios (IRRs) for 30 extra-colorectal malignancies with comparison to the general population.Results:Significantly increased IRRs were identified for 13 cancer types with differences related to gender, age and disease-predisposing gene. The different cancer types showed variable peak age incidence rates (IRs) with the highest IRs for ovarian cancer at age 30-49 years, for endometrial cancer, breast cancer, renal cell cancer and brain tumours at age 50-69 years, and for urothelial cancer, small bowel cancer, gastric cancer, pancreatic cancer and skin tumours after age 70.Conclusions:The broad spectrum of tumour types that develop at an increased incidence defines Lynch syndrome as a multi-tumour syndrome. The variable incidences in relation to age, gender and gene suggest a need for individualised surveillance.</p>},
  author       = {Therkildsen, Christina and Ladelund, Steen and Smith-Hansen, Lars and Lindberg, Lars Joachim and Nilbert, Mef},
  issn         = {0007-0920},
  keyword      = {breast cancer,colorectal cancer,hereditary non-polyposis colorectal cancer,mismatch repair genes,pancreatic cancer,prostate cancer},
  language     = {eng},
  month        = {11},
  number       = {11},
  pages        = {1702--1710},
  publisher    = {Nature Publishing Group},
  series       = {British Journal of Cancer},
  title        = {Towards gene-and gender-based risk estimates in Lynch syndrome; Age-specific incidences for 13 extra-colorectal cancer types},
  url          = {http://dx.doi.org/10.1038/bjc.2017.348},
  volume       = {117},
  year         = {2017},
}