Towards gene-and gender-based risk estimates in Lynch syndrome; Age-specific incidences for 13 extra-colorectal cancer types
(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
- Therkildsen, Christina LU ; Ladelund, Steen ; Smith-Hansen, Lars ; Lindberg, Lars Joachim and Nilbert, Mef LU
- organization
- publishing date
- 2017-11-21
- 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
- pmid:29065108
- 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
- 2025-01-21 04:52:39
@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}},
keywords = {{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}},
doi = {{10.1038/bjc.2017.348}},
volume = {{117}},
year = {{2017}},
}