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@ARTICLE{Lefler:302807,
author = {D. S. Lefler and A. Elliott and W. Jiang and U.
Martinez-Outschoorn and J. Al-Sabah and D. M. Freed and C.
M. King and R. G. Maki and R. F. Riedel and J. F. Modiano
and D. Hübschmann$^*$ and H. Glimm$^*$ and S. Fröhling$^*$
and M. Oberley and S. A. Boikos and A. Basu Mallick},
title = {{P}ansarcoma {A}nalysis of {C}yclin-{D}ependent {K}inase
and {C}yclin {O}utlier {G}ene {E}xpression {H}ighlights
{CDK}7 as a {P}otential {T}herapeutic {T}arget in
{C}hordoma},
journal = {JCO precision oncology},
volume = {9},
issn = {2473-4284},
address = {Alexandria, VA},
publisher = {American Society of Clinical Oncology},
reportid = {DKFZ-2025-01347},
pages = {e2500149},
year = {2025},
note = {2025 Jul:9:e2500149},
abstract = {Purpose: Cyclin-dependent kinase (CDK)4/6 inhibitors are
approved for the treatment of breast cancer, and they have
more recently been used in patients with
well-differentiated/dedifferentiated liposarcomas
(WD-LPSs/DD-LPSs). However, targeting of these and other
CDKs, including transcriptional CDKs, remains a promising
avenue of investigation for various cancers. Therefore, we
sought to characterize outlier overexpression of CDK and
cyclin genes in sarcomas. On the basis of the initial
results, further studies were undertaken to investigate the
roles of CDK7 and CDK18 in chordomas.Materials and methods:
An initial analysis of CDK/cyclin gene expression involved
an American national biomarker database of deidentified
patients (Caris Life Sciences, Phoenix, AZ; n = 3,757) using
novel, strict definitions to identify outlier overexpressing
samples across subtypes. Results were validated with a
German national database (Molecularly Aided Stratification
for Tumor Eradication Research [MASTER]; n = 943). Outlier
overexpression for CDK7/18 in chordoma was compared with
immunohistochemical (IHC) expression using tissue
microarrays, and a selective investigational CDK7 inhibitor
was tested against four chordoma cell lines.Results: Initial
analysis identified expected findings (eg, outlier
overexpression of CDK4 in $39\%-66\%$ of WD-LPSs/DD-LPSs),
clinical correlates of fundamental scientific work (eg,
CCND1 in $29\%$ of Ewing sarcomas), and novel associations
(eg, CDK7/CDK18 in $42\%/37\%$ of chordomas). Outlier
overexpression for CDK7 and CDK18 in chordomas was
corroborated in the MASTER database $(40\%$ and $26\%$ of
patients, respectively). IHC analysis confirmed strong and
diffuse expression of both CDK7 and CDK18 in chordoma
samples. Furthermore, CDK7 inhibition was highly effective
in four chordoma cell lines.Conclusion: This study supports
further investigation into targeting of CDKs and cyclins in
select sarcoma subtypes, and it specifically suggests a
therapeutic approach inhibiting CDK7 in chordoma.},
cin = {W015 / HD01 / B340 / B280},
ddc = {610},
cid = {I:(DE-He78)W015-20160331 / I:(DE-He78)HD01-20160331 /
I:(DE-He78)B340-20160331 / I:(DE-He78)B280-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40601892},
doi = {10.1200/PO-25-00149},
url = {https://inrepo02.dkfz.de/record/302807},
}