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@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},
}