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@ARTICLE{Achatz:300326,
author = {M. I. Achatz and A. Villani and A. A. Bertuch and G.
Bougeard and V. Y. Chang and A. S. Doria and B. Gallinger
and L. A. Godley and M. C. Greer and J. Kamihara and P. P.
Khincha and W. K. Kohlmann and C. P. Kratz and S. P.
MacFarland and L. D. Maese and K. N. Maxwell and S. G.
Mitchell and Y. Nakano and S. Pfister$^*$ and J. D.
Wasserman and E. R. Woodward and J. E. Garber and D. Malkin},
title = {{U}pdate on {C}ancer {S}creening {R}ecommendations for
{I}ndividuals with {L}i-{F}raumeni {S}yndrome.},
journal = {Clinical cancer research},
volume = {31},
number = {10},
issn = {1078-0432},
address = {Philadelphia, Pa. [u.a.]},
publisher = {AACR},
reportid = {DKFZ-2025-00772},
pages = {1831-1840},
year = {2025},
note = {2025 May 15;31(10):1831-1840 / Perspective},
abstract = {Li-Fraumeni syndrome (LFS) is an autosomal dominant cancer
predisposition condition characterized by a high lifetime
risk for a wide spectrum of malignancies associated with
germline pathogenic/likely pathogenic (P/LP) variants in the
TP53 tumor suppressor gene. Secondary malignant neoplasms
are particularly common. Early cancer detection through
surveillance enables early intervention and leads to
improved clinical outcomes with reduced tumor-related
mortality and treatment-related morbidity. Since the 2017
publication of LFS tumor surveillance guidelines from the
inaugural AACR Childhood Cancer Predisposition Workshop,
understanding the genotype:phenotype relationships in LFS
have evolved, and adaptations of the guidelines have been
implemented in institutions worldwide. The 'Toronto
Protocol' remains the current standard for life-long
surveillance; however, as outlined in this Perspective,
modifications should be considered as to the use of certain
modalities to target organs in an age-dependent manner. The
Working Group's recommendations have also been extended to
include a more detailed outline for surveillance in the
adult TP53 P/LP variant carrier population based on the
recognition that early education of both practitioners and
patients on what to expect after the transition from
childhood/adolescence to young adulthood is important in
preparing them for changes in surveillance strategies. In
this perspective, we provide an up-to-date clinical overview
of LFS, and present our updated consensus tumor surveillance
recommendations from the 2023 AACR Childhood Cancer
Predisposition Workshop.},
subtyp = {Review Article},
cin = {B062 / HD01},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40072304},
doi = {10.1158/1078-0432.CCR-24-3301},
url = {https://inrepo02.dkfz.de/record/300326},
}