% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Kratz:124288,
author = {C. P. Kratz and M. I. Achatz and L. Brugières and T.
Frebourg and J. E. Garber and M. C. Greer and J. R. Hansford
and K. A. Janeway and W. K. Kohlmann and R. McGee and C. G.
Mullighan and K. Onel and K. Pajtler$^*$ and S. Pfister$^*$
and S. A. Savage and J. D. Schiffman and K. A. Schneider and
L. C. Strong and D. G. R. Evans and J. D. Wasserman and A.
Villani and D. Malkin},
title = {{C}ancer {S}creening {R}ecommendations for {I}ndividuals
with {L}i-{F}raumeni {S}yndrome.},
journal = {Clinical cancer research},
volume = {23},
number = {11},
issn = {1557-3265},
address = {Philadelphia, Pa. [u.a.]},
publisher = {AACR},
reportid = {DKFZ-2017-01184},
pages = {e38 - e45},
year = {2017},
abstract = {Li-Fraumeni syndrome (LFS) is an autosomal dominantly
inherited condition caused by germline mutations of the TP53
tumor suppressor gene encoding p53, a transcription factor
triggered as a protective cellular mechanism against
different stressors. Loss of p53 function renders affected
individuals highly susceptible to a broad range of solid and
hematologic cancers. It has recently become evident that
children and adults with LFS benefit from intensive
surveillance aimed at early tumor detection. In October
2016, the American Association for Cancer Research held a
meeting of international LFS experts to evaluate the current
knowledge on LFS and propose consensus surveillance
recommendations. Herein, we briefly summarize clinical and
genetic aspects of this aggressive cancer predisposition
syndrome. In addition, the expert panel concludes that there
are sufficient existing data to recommend that all patients
with LFS be offered cancer surveillance as soon as the
clinical or molecular LFS diagnosis is established.
Specifically, the panel recommends adoption of a modified
version of the 'Toronto protocol' that includes a
combination of physical exams, blood tests, and imaging. The
panel also recommends that further research be promoted to
explore the feasibility and effectiveness of these
risk-adapted surveillance and cancer prevention strategies
while addressing the psychosocial needs of individuals and
families with LFS. Clin Cancer Res; 23(11); e38-e45. ©2017
AACRSee all articles in the online-only CCR Pediatric
Oncology Series.},
subtyp = {Review Article},
cin = {B062},
ddc = {610},
cid = {I:(DE-He78)B062-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:28572266},
doi = {10.1158/1078-0432.CCR-17-0408},
url = {https://inrepo02.dkfz.de/record/124288},
}