Cancer Screening Recommendations for Individuals with Li-Fraumeni Syndrome.
Identifieur interne : 001057 ( Main/Exploration ); précédent : 001056; suivant : 001058Cancer Screening Recommendations for Individuals with Li-Fraumeni Syndrome.
Auteurs : Christian P. Kratz [Allemagne] ; Maria Isabel Achatz [États-Unis] ; Laurence Brugières [France] ; Thierry Frebourg [France] ; Judy E. Garber [États-Unis] ; Mary-Louise C. Greer [Canada] ; Jordan R. Hansford [Australie] ; Katherine A. Janeway [États-Unis] ; Wendy K. Kohlmann [États-Unis] ; Rose Mcgee [États-Unis] ; Charles G. Mullighan [États-Unis] ; Kenan Onel [États-Unis] ; Kristian W. Pajtler [Allemagne] ; Stefan M. Pfister [Allemagne] ; Sharon A. Savage [États-Unis] ; Joshua D. Schiffman [États-Unis] ; Katherine A. Schneider [États-Unis] ; Louise C. Strong [États-Unis] ; D Gareth R. Evans [Royaume-Uni] ; Jonathan D. Wasserman [Royaume-Uni] ; Anita Villani [Canada] ; David Malkin [Canada]Source :
- Clinical cancer research : an official journal of the American Association for Cancer Research [ 1078-0432 ] ; 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.
DOI: 10.1158/1078-0432.CCR-17-0408
PubMed: 28572266
Affiliations:
- Allemagne, Australie, Canada, France, Royaume-Uni, États-Unis
- Angleterre, Bade-Wurtemberg, Basse-Saxe, District de Karlsruhe, Grand Manchester, Haute-Normandie, Maryland, Massachusetts, Ontario, Région Normandie, Tennessee, Texas, Utah, Victoria (État), État de New York
- Hanovre, Heidelberg, Manchester, Melbourne, Rouen, Toronto
- Université de Manchester, Université de Melbourne, Université de Toronto
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<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">Tennessee</region>
</placeName>
<wicri:cityArea>Department of Pathology, St. Jude Children's Research Hospital, Memphis</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Onel, Kenan" sort="Onel, Kenan" uniqKey="Onel K" first="Kenan" last="Onel">Kenan Onel</name>
<affiliation wicri:level="2"><nlm:affiliation>Hofstra Northwell School of Medicine, Cohen Children's Medical Center, Northwell Health, Manhasset, New York.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">État de New York</region>
</placeName>
<wicri:cityArea>Hofstra Northwell School of Medicine, Cohen Children's Medical Center, Northwell Health, Manhasset</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Pajtler, Kristian W" sort="Pajtler, Kristian W" uniqKey="Pajtler K" first="Kristian W" last="Pajtler">Kristian W. Pajtler</name>
<affiliation wicri:level="3"><nlm:affiliation>Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg</wicri:regionArea>
<placeName><region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Karlsruhe</region>
<settlement type="city">Heidelberg</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Pfister, Stefan M" sort="Pfister, Stefan M" uniqKey="Pfister S" first="Stefan M" last="Pfister">Stefan M. Pfister</name>
<affiliation wicri:level="3"><nlm:affiliation>Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg</wicri:regionArea>
<placeName><region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Karlsruhe</region>
<settlement type="city">Heidelberg</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Savage, Sharon A" sort="Savage, Sharon A" uniqKey="Savage S" first="Sharon A" last="Savage">Sharon A. Savage</name>
<affiliation wicri:level="2"><nlm:affiliation>Clinical Genetics Branch, National Cancer Institute, Bethesda, Maryland.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">Maryland</region>
</placeName>
<wicri:cityArea>Clinical Genetics Branch, National Cancer Institute, Bethesda</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Schiffman, Joshua D" sort="Schiffman, Joshua D" uniqKey="Schiffman J" first="Joshua D" last="Schiffman">Joshua D. Schiffman</name>
<affiliation wicri:level="2"><nlm:affiliation>Department of Pediatrics and Huntsman Cancer Institute, University of Utah, Salt Lake City, Utah.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">Utah</region>
</placeName>
<wicri:cityArea>Department of Pediatrics and Huntsman Cancer Institute, University of Utah, Salt Lake City</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Schneider, Katherine A" sort="Schneider, Katherine A" uniqKey="Schneider K" first="Katherine A" last="Schneider">Katherine A. Schneider</name>
<affiliation wicri:level="2"><nlm:affiliation>Center for Cancer Genetics and Prevention, Dana-Farber Cancer Institute, Boston, Massachusetts.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">Massachusetts</region>
</placeName>
<wicri:cityArea>Center for Cancer Genetics and Prevention, Dana-Farber Cancer Institute, Boston</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Strong, Louise C" sort="Strong, Louise C" uniqKey="Strong L" first="Louise C" last="Strong">Louise C. Strong</name>
<affiliation wicri:level="2"><nlm:affiliation>Department of Genetics, The University of Texas MD Anderson Cancer Center, Houston, Texas.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<placeName><region type="state">Texas</region>
</placeName>
<wicri:cityArea>Department of Genetics, The University of Texas MD Anderson Cancer Center, Houston</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Evans, D Gareth R" sort="Evans, D Gareth R" uniqKey="Evans D" first="D Gareth R" last="Evans">D Gareth R. Evans</name>
<affiliation wicri:level="4"><nlm:affiliation>Medical Genetics and Cancer Epidemiology, Genomic Medicine, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Medical Genetics and Cancer Epidemiology, Genomic Medicine, Manchester Academic Health Science Centre, University of Manchester, Manchester</wicri:regionArea>
<placeName><settlement type="city">Manchester</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Manchester</region>
<settlement type="city">Manchester</settlement>
</placeName>
<orgName type="university">Université de Manchester</orgName>
</affiliation>
</author>
<author><name sortKey="Wasserman, Jonathan D" sort="Wasserman, Jonathan D" uniqKey="Wasserman J" first="Jonathan D" last="Wasserman">Jonathan D. Wasserman</name>
<affiliation wicri:level="4"><nlm:affiliation>Medical Genetics and Cancer Epidemiology, Genomic Medicine, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Medical Genetics and Cancer Epidemiology, Genomic Medicine, Manchester Academic Health Science Centre, University of Manchester, Manchester</wicri:regionArea>
<placeName><settlement type="city">Manchester</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Manchester</region>
<settlement type="city">Manchester</settlement>
</placeName>
<orgName type="university">Université de Manchester</orgName>
</affiliation>
</author>
<author><name sortKey="Villani, Anita" sort="Villani, Anita" uniqKey="Villani A" first="Anita" last="Villani">Anita Villani</name>
<affiliation wicri:level="4"><nlm:affiliation>Division of Endocrinology, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada. david.malkin@sickkids.ca anita.villani@sickkids.ca.</nlm:affiliation>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>Division of Endocrinology, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario</wicri:regionArea>
<orgName type="university">Université de Toronto</orgName>
<placeName><settlement type="city">Toronto</settlement>
<region type="state">Ontario</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Malkin, David" sort="Malkin, David" uniqKey="Malkin D" first="David" last="Malkin">David Malkin</name>
<affiliation wicri:level="4"><nlm:affiliation>Division of Endocrinology, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada. david.malkin@sickkids.ca anita.villani@sickkids.ca.</nlm:affiliation>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>Division of Endocrinology, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario</wicri:regionArea>
<orgName type="university">Université de Toronto</orgName>
<placeName><settlement type="city">Toronto</settlement>
<region type="state">Ontario</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">Clinical cancer research : an official journal of the American Association for Cancer Research</title>
<idno type="ISSN">1078-0432</idno>
<imprint><date when="2017" type="published">2017</date>
</imprint>
</series>
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<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">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.</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Australie</li>
<li>Canada</li>
<li>France</li>
<li>Royaume-Uni</li>
<li>États-Unis</li>
</country>
<region><li>Angleterre</li>
<li>Bade-Wurtemberg</li>
<li>Basse-Saxe</li>
<li>District de Karlsruhe</li>
<li>Grand Manchester</li>
<li>Haute-Normandie</li>
<li>Maryland</li>
<li>Massachusetts</li>
<li>Ontario</li>
<li>Région Normandie</li>
<li>Tennessee</li>
<li>Texas</li>
<li>Utah</li>
<li>Victoria (État)</li>
<li>État de New York</li>
</region>
<settlement><li>Hanovre</li>
<li>Heidelberg</li>
<li>Manchester</li>
<li>Melbourne</li>
<li>Rouen</li>
<li>Toronto</li>
</settlement>
<orgName><li>Université de Manchester</li>
<li>Université de Melbourne</li>
<li>Université de Toronto</li>
</orgName>
</list>
<tree><country name="Allemagne"><region name="Basse-Saxe"><name sortKey="Kratz, Christian P" sort="Kratz, Christian P" uniqKey="Kratz C" first="Christian P" last="Kratz">Christian P. Kratz</name>
</region>
<name sortKey="Pajtler, Kristian W" sort="Pajtler, Kristian W" uniqKey="Pajtler K" first="Kristian W" last="Pajtler">Kristian W. Pajtler</name>
<name sortKey="Pfister, Stefan M" sort="Pfister, Stefan M" uniqKey="Pfister S" first="Stefan M" last="Pfister">Stefan M. Pfister</name>
</country>
<country name="États-Unis"><region name="Maryland"><name sortKey="Achatz, Maria Isabel" sort="Achatz, Maria Isabel" uniqKey="Achatz M" first="Maria Isabel" last="Achatz">Maria Isabel Achatz</name>
</region>
<name sortKey="Garber, Judy E" sort="Garber, Judy E" uniqKey="Garber J" first="Judy E" last="Garber">Judy E. Garber</name>
<name sortKey="Janeway, Katherine A" sort="Janeway, Katherine A" uniqKey="Janeway K" first="Katherine A" last="Janeway">Katherine A. Janeway</name>
<name sortKey="Kohlmann, Wendy K" sort="Kohlmann, Wendy K" uniqKey="Kohlmann W" first="Wendy K" last="Kohlmann">Wendy K. Kohlmann</name>
<name sortKey="Mcgee, Rose" sort="Mcgee, Rose" uniqKey="Mcgee R" first="Rose" last="Mcgee">Rose Mcgee</name>
<name sortKey="Mullighan, Charles G" sort="Mullighan, Charles G" uniqKey="Mullighan C" first="Charles G" last="Mullighan">Charles G. Mullighan</name>
<name sortKey="Onel, Kenan" sort="Onel, Kenan" uniqKey="Onel K" first="Kenan" last="Onel">Kenan Onel</name>
<name sortKey="Savage, Sharon A" sort="Savage, Sharon A" uniqKey="Savage S" first="Sharon A" last="Savage">Sharon A. Savage</name>
<name sortKey="Schiffman, Joshua D" sort="Schiffman, Joshua D" uniqKey="Schiffman J" first="Joshua D" last="Schiffman">Joshua D. Schiffman</name>
<name sortKey="Schneider, Katherine A" sort="Schneider, Katherine A" uniqKey="Schneider K" first="Katherine A" last="Schneider">Katherine A. Schneider</name>
<name sortKey="Strong, Louise C" sort="Strong, Louise C" uniqKey="Strong L" first="Louise C" last="Strong">Louise C. Strong</name>
</country>
<country name="France"><noRegion><name sortKey="Brugieres, Laurence" sort="Brugieres, Laurence" uniqKey="Brugieres L" first="Laurence" last="Brugières">Laurence Brugières</name>
</noRegion>
<name sortKey="Frebourg, Thierry" sort="Frebourg, Thierry" uniqKey="Frebourg T" first="Thierry" last="Frebourg">Thierry Frebourg</name>
</country>
<country name="Canada"><region name="Ontario"><name sortKey="Greer, Mary Louise C" sort="Greer, Mary Louise C" uniqKey="Greer M" first="Mary-Louise C" last="Greer">Mary-Louise C. Greer</name>
</region>
<name sortKey="Malkin, David" sort="Malkin, David" uniqKey="Malkin D" first="David" last="Malkin">David Malkin</name>
<name sortKey="Villani, Anita" sort="Villani, Anita" uniqKey="Villani A" first="Anita" last="Villani">Anita Villani</name>
</country>
<country name="Australie"><region name="Victoria (État)"><name sortKey="Hansford, Jordan R" sort="Hansford, Jordan R" uniqKey="Hansford J" first="Jordan R" last="Hansford">Jordan R. Hansford</name>
</region>
</country>
<country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Evans, D Gareth R" sort="Evans, D Gareth R" uniqKey="Evans D" first="D Gareth R" last="Evans">D Gareth R. Evans</name>
</region>
<name sortKey="Wasserman, Jonathan D" sort="Wasserman, Jonathan D" uniqKey="Wasserman J" first="Jonathan D" last="Wasserman">Jonathan D. Wasserman</name>
</country>
</tree>
</affiliations>
</record>
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