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@ARTICLE{Ahadova:134800,
author = {A. Ahadova$^*$ and R. Gallon and J. Gebert$^*$ and A.
Ballhausen$^*$ and V. Endris and M. Kirchner and A.
Stenzinger and J. Burn and M. von Knebel Doeberitz$^*$ and
H. Bläker and M. Kloor$^*$},
title = {{T}hree molecular pathways model colorectal carcinogenesis
in {L}ynch syndrome.},
journal = {International journal of cancer},
volume = {143},
number = {1},
issn = {0020-7136},
address = {Bognor Regis},
publisher = {Wiley-Liss},
reportid = {DKFZ-2018-00593},
pages = {139 - 150},
year = {2018},
abstract = {Lynch syndrome is caused by germline mutations of DNA
mismatch repair (MMR) genes. MMR deficiency has long been
regarded as a secondary event in the pathogenesis of Lynch
syndrome colorectal cancers. Recently, this concept has been
challenged by the discovery of MMR-deficient crypt foci in
the normal mucosa. We aimed to reconstruct colorectal
carcinogenesis in Lynch syndrome by collecting molecular and
histology evidence from Lynch syndrome adenomas and
carcinomas. We determined the frequency of MMR deficiency in
adenomas from Lynch syndrome mutation carriers by
immunohistochemistry and by systematic literature analysis.
To trace back the pathways of pathogenesis, histological
growth patterns and mutational signatures were analyzed in
Lynch syndrome colorectal cancers. Literature and
immunohistochemistry analysis demonstrated MMR deficiency in
491 $(76.7\%)$ out of 640 adenomas $(95\%$ CI: $73.3\%$ to
$79.8\%)$ from Lynch syndrome mutation carriers.
Histologically normal MMR-deficient crypts were found
directly adjacent to dysplastic adenoma tissue, proving
their role as tumor precursors in Lynch syndrome.
Accordingly, mutation signature analysis in Lynch colorectal
cancers revealed that KRAS and APC mutations commonly occur
after the onset of MMR deficiency. Tumors lacking evidence
of polypous growth frequently presented with CTNNB1 and TP53
mutations. Our findings demonstrate that Lynch syndrome
colorectal cancers can develop through three pathways, with
MMR deficiency commonly representing an early and possibly
initiating event. This underlines that targeting
MMR-deficient cells by chemoprevention or vaccines against
MMR deficiency-induced frameshift peptide neoantigens holds
promise for tumor prevention in Lynch syndrome.},
cin = {G105},
ddc = {610},
cid = {I:(DE-He78)G105-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:29424427},
doi = {10.1002/ijc.31300},
url = {https://inrepo02.dkfz.de/record/134800},
}