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@ARTICLE{Lffler:129069,
author = {H. Löffler$^*$ and N. Pfarr and M. Kriegsmann and V.
Endris and T. Hielscher$^*$ and P. Lohneis and G. Folprecht
and A. Stenzinger and M. Dietel and W. Weichert$^*$ and A.
Krämer$^*$},
title = {{M}olecular driver alterations and their clinical relevance
in cancer of unknown primary site.},
journal = {OncoTarget},
volume = {7},
number = {28},
issn = {1949-2553},
address = {[S.l.]},
publisher = {Impact Journals LLC},
reportid = {DKFZ-2017-05074},
pages = {44322 - 44329},
year = {2016},
abstract = {Cancer of unknown primary (CUP) is defined as metastatic
solid malignancy where no primary tumor is detected despite
appropriate staging. About $90\%$ of CUP represent
adenocarcinoma or undifferentiated carcinoma. Since therapy
regimens are only modestly effective, identification of the
molecular landscape of these neoplasms might be a promising
approach to direct CUP therapy and aid in tumor
classification. We screened a cohort of 128 patients with
adenocarcinoma or undifferentiated carcinoma meeting the
definition of CUP. Massive parallel multigene sequencing of
50 genes, which had been selected due to their relevance as
oncogenic drivers or druggable molecular targets could
ultimately be performed on samples from 55 patients for whom
complete clinical datasets were also available. Overall, 60
tumor-specific mutations and 29 amplifications/deletions, as
revealed by coverage analysis, were detected in 46 cases
$(84\%).$ The most frequently mutated genes were TP53 (30
cases, $55\%),$ KRAS (9 cases, $16\%),$ CDKN2A (5 cases,
$9\%),$ and SMAD4 (5 cases, $9\%).$ The most frequently
deleted gene was CDKN2A (8 cases, $15\%).$ KRAS and CDKN2A
mutations significantly correlated with poor
progression-free survival (PFS) and, in case of KRAS,
overall survival (OS). WIldtype TP53 and female sex defined
a relatively favorable category, with favorable PFS and OS.
8 cases $(15\%)$ harbored mutations that may be targetable
by currently approved drugs. Taken together, Mutations of
relevant driver genes are present in the vast majority of
CUP tumors. Some of them impact on prognosis and a subset is
putatively druggable.},
cin = {G330 / C060 / L101 / L701 / L201 / L301},
ddc = {610},
cid = {I:(DE-He78)G330-20160331 / I:(DE-He78)C060-20160331 /
I:(DE-He78)L101-20160331 / I:(DE-He78)L701-20160331 /
I:(DE-He78)L201-20160331 / I:(DE-He78)L301-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:27322425},
pmc = {pmc:PMC5190099},
doi = {10.18632/oncotarget.10035},
url = {https://inrepo02.dkfz.de/record/129069},
}