Frequency and spectrum of cancers in the peutz-jeghers syndrome
Identifieur interne : 009F21 ( Main/Exploration ); précédent : 009F20; suivant : 009F22Frequency and spectrum of cancers in the peutz-jeghers syndrome
Auteurs : Nicholas Hearle [Royaume-Uni] ; Valerie Schumacher [Allemagne] ; Fred H. Menko [Pays-Bas] ; Sylviane Olschwang [France] ; Lisa A. Boardman [États-Unis] ; Johan J. P. Gille [Pays-Bas] ; Josbert J. Keller [Pays-Bas] ; Anne Marie Westerman [Pays-Bas] ; Rodney J. Scott [Australie] ; Wendy Lim [Royaume-Uni] ; Jill D. Trimbath [États-Unis] ; Francis M. Giardiello [États-Unis] ; Stephen B. Gruber [États-Unis] ; G. Johan A. Offerhaus [Pays-Bas] ; Felix W. M. De Rooij [Pays-Bas] ; J. H. Paul Wilson [Pays-Bas] ; Anika Hansmann [Allemagne] ; Gabriela Möslein [Allemagne] ; Brigitte Royer-Pokora [Allemagne] ; TILMANVOGEL [Allemagne] ; Robin K. S. Phillips [Royaume-Uni] ; Allan D. Spigelman [Australie] ; Richard S. Houlston [Royaume-Uni]Source :
- Clinical cancer research [ 1078-0432 ] ; 2006.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
Abstract
Background: Although an increased cancer risk in Peutz-Jeghers syndrome is established, data on the spectrum of tumors associated with the disease and the influence of germ-line STK11I LKB1 (serine/threonine kinase) mutation status are limited. Experimental Design: We analyzed the incidence of cancer in 419 individuals with Peutz-Jeghers syndrome, and 297 had documented STK11/LKB1 mutations. Results: Ninety-six cancers were found among individuals with Peutz-Jeghers syndrome. The risk for developing cancer at ages 20, 30, 40, 50, 60, and 70 years was 2%, 6%, 17%, 31%, 60%, and 85%, respectively. The most common cancers represented in this analysis were gastrointestinal in origin, gastroesophageal, small bowel, colorectal, and pancreatic, and the risk for these cancers at ages 30, 40, 50, and 60 years was 1%, 9%, 15%, and 33%, respectively. In women with Peutz-Jeghers syndrome, the risk of breast cancer was substantially increased, being 8% and 31% at ages 40 and 60 years, respectively. Kaplan-Meier analysis showed that cancer risks were similar in Peutz-Jeghers syndrome patients with identified STK11/LKB1 mutations and those with no detectable mutation (log-rank test of difference X2 = 0.62; 1 df; P = 0.43). Furthermore, the type or site of STK11/LKB1 mutation did not significantly influence cancer risk. Conclusions: The results from our study provide quantitative information on the spectrum of cancers and risks of specific cancer types associated with Peutz-Jeghers syndrome.
Affiliations:
- Allemagne, Australie, France, Pays-Bas, Royaume-Uni, États-Unis
- District de Düsseldorf, Hollande-Méridionale, Hollande-Septentrionale, Maryland, Michigan, Minnesota, Rhénanie-du-Nord-Westphalie
- Amsterdam, Düsseldorf, Rotterdam
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Boardman</name><affiliation wicri:level="2"><inist:fA14 i1="05"><s1>Division of Gastroenterology, Department of Internal Medicine, Mayo Clinic, Mayo Foundation</s1><s2>Rochester, Minnesota</s2><s3>USA</s3><sZ>5 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Minnesota</region></placeName></affiliation></author><author><name sortKey="Gille, Johan J P" sort="Gille, Johan J P" uniqKey="Gille J" first="Johan J. P." last="Gille">Johan J. P. Gille</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</s1><s3>NLD</s3><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Pays-Bas</country><wicri:noRegion>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</wicri:noRegion></affiliation></author><author><name sortKey="Keller, Josbert J" sort="Keller, Josbert J" uniqKey="Keller J" first="Josbert J." last="Keller">Josbert J. Keller</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Pathology, Academic Medical Centre</s1><s2>Amsterdam</s2><s3>NLD</s3><sZ>7 aut.</sZ><sZ>14 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region></placeName></affiliation></author><author><name sortKey="Westerman, Anne Marie" sort="Westerman, Anne Marie" uniqKey="Westerman A" first="Anne Marie" last="Westerman">Anne Marie Westerman</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Scott, Rodney J" sort="Scott, Rodney J" uniqKey="Scott R" first="Rodney J." last="Scott">Rodney J. Scott</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Discipline of Medical Genetics, Faculty of Health, Newcastle and Hunter Medical Research Institute</s1><s2>Newcastle, New South Wales</s2><s3>AUS</s3><sZ>9 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Newcastle, New South Wales</wicri:noRegion></affiliation></author><author><name sortKey="Lim, Wendy" sort="Lim, Wendy" uniqKey="Lim W" first="Wendy" last="Lim">Wendy Lim</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Section of Cancer Genetics, Institute of Cancer Research</s1><s2>Sutton</s2><s3>GBR</s3><sZ>1 aut.</sZ><sZ>10 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Sutton</wicri:noRegion></affiliation></author><author><name sortKey="Trimbath, Jill D" sort="Trimbath, Jill D" uniqKey="Trimbath J" first="Jill D." last="Trimbath">Jill D. Trimbath</name><affiliation wicri:level="2"><inist:fA14 i1="09"><s1>Department of Medicine, Johns Hopkins University School of Medicine</s1><s2>Baltimore, Maryland</s2><s3>USA</s3><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Giardiello, Francis M" sort="Giardiello, Francis M" uniqKey="Giardiello F" first="Francis M." last="Giardiello">Francis M. Giardiello</name><affiliation wicri:level="2"><inist:fA14 i1="09"><s1>Department of Medicine, Johns Hopkins University School of Medicine</s1><s2>Baltimore, Maryland</s2><s3>USA</s3><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Gruber, Stephen B" sort="Gruber, Stephen B" uniqKey="Gruber S" first="Stephen B." last="Gruber">Stephen B. Gruber</name><affiliation wicri:level="2"><inist:fA14 i1="10"><s1>Division of Molecular Medicine and Genetics, University of Michigan</s1><s2>Ann Arbor, Michigan</s2><s3>USA</s3><sZ>13 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Michigan</region></placeName></affiliation></author><author><name sortKey="Offerhaus, G Johan A" sort="Offerhaus, G Johan A" uniqKey="Offerhaus G" first="G. Johan A." last="Offerhaus">G. Johan A. Offerhaus</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Pathology, Academic Medical Centre</s1><s2>Amsterdam</s2><s3>NLD</s3><sZ>7 aut.</sZ><sZ>14 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region></placeName></affiliation></author><author><name sortKey="De Rooij, Felix W M" sort="De Rooij, Felix W M" uniqKey="De Rooij F" first="Felix W. M." last="De Rooij">Felix W. M. De Rooij</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Wilson, J H Paul" sort="Wilson, J H Paul" uniqKey="Wilson J" first="J. H. Paul" last="Wilson">J. H. Paul Wilson</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Hansmann, Anika" sort="Hansmann, Anika" uniqKey="Hansmann A" first="Anika" last="Hansmann">Anika Hansmann</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Institute of Human Genetics, Heinrich-Heine University</s1><s2>Düsseldorf</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>17 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Düsseldorf</region><settlement type="city">Düsseldorf</settlement></placeName></affiliation></author><author><name sortKey="Moslein, Gabriela" sort="Moslein, Gabriela" uniqKey="Moslein G" first="Gabriela" last="Möslein">Gabriela Möslein</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of General and Visceral Surgery, St. Josefs-Hospital Bochum-Linden</s1><s2>Bochum</s2><s3>DEU</s3><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Bochum</wicri:noRegion><wicri:noRegion>St. Josefs-Hospital Bochum-Linden</wicri:noRegion><wicri:noRegion>Bochum</wicri:noRegion></affiliation></author><author><name sortKey="Royer Pokora, Brigitte" sort="Royer Pokora, Brigitte" uniqKey="Royer Pokora B" first="Brigitte" last="Royer-Pokora">Brigitte Royer-Pokora</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Institute of Human Genetics, Heinrich-Heine University</s1><s2>Düsseldorf</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>17 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Düsseldorf</region><settlement type="city">Düsseldorf</settlement></placeName></affiliation></author><author><name sortKey="Tilmanvogel" sort="Tilmanvogel" uniqKey="Tilmanvogel" last="Tilmanvogel">TILMANVOGEL</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Department of General, Visceral and Thoracic Surgery, Klinken Maria Hilf</s1><s2>Mönchengladbach</s2><s3>DEU</s3><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Mönchengladbach</wicri:noRegion><wicri:noRegion>Klinken Maria Hilf</wicri:noRegion><wicri:noRegion>Mönchengladbach</wicri:noRegion></affiliation></author><author><name sortKey="Phillips, Robin K S" sort="Phillips, Robin K S" uniqKey="Phillips R" first="Robin K. S." last="Phillips">Robin K. S. Phillips</name><affiliation wicri:level="1"><inist:fA14 i1="13"><s1>Polyposis Registry, St. Mark's Hospital</s1><s2>Harrow</s2><s3>GBR</s3><sZ>21 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Harrow</wicri:noRegion></affiliation></author><author><name sortKey="Spigelman, Allan D" sort="Spigelman, Allan D" uniqKey="Spigelman A" first="Allan D." last="Spigelman">Allan D. Spigelman</name><affiliation wicri:level="1"><inist:fA14 i1="14"><s1>Professorial Surgical Unit, Faculty of Medicine, St. Vincent's Hospital Clinical School, University of New South Wales</s1><s3>AUS</s3><sZ>22 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Professorial Surgical Unit, Faculty of Medicine, St. Vincent's Hospital Clinical School, University of New South Wales</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Hunter Family Cancer Service and New South Wales and Australian Capital Territory Hereditary Cancer Registries</s1><s2>Sydney, New South Wales</s2><s3>AUS</s3><sZ>22 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Hunter Family Cancer Service and New South Wales and Australian Capital Territory Hereditary Cancer Registries</wicri:noRegion></affiliation></author><author><name sortKey="Houlston, Richard S" sort="Houlston, Richard S" uniqKey="Houlston R" first="Richard S." last="Houlston">Richard S. Houlston</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Section of Cancer Genetics, Institute of Cancer Research</s1><s2>Sutton</s2><s3>GBR</s3><sZ>1 aut.</sZ><sZ>10 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Sutton</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">06-0368710</idno><date when="2006">2006</date><idno type="stanalyst">PASCAL 06-0368710 INIST</idno><idno type="RBID">Pascal:06-0368710</idno><idno type="wicri:Area/PascalFrancis/Corpus">004277</idno><idno type="wicri:Area/PascalFrancis/Curation">001E18</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">004016</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">004016</idno><idno type="wicri:doubleKey">1078-0432:2006:Hearle N:frequency:and:spectrum</idno><idno type="wicri:Area/Main/Merge">00AA38</idno><idno type="wicri:Area/Main/Curation">009F21</idno><idno type="wicri:Area/Main/Exploration">009F21</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Frequency and spectrum of cancers in the peutz-jeghers syndrome</title><author><name sortKey="Hearle, Nicholas" sort="Hearle, Nicholas" uniqKey="Hearle N" first="Nicholas" last="Hearle">Nicholas Hearle</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Section of Cancer Genetics, Institute of Cancer Research</s1><s2>Sutton</s2><s3>GBR</s3><sZ>1 aut.</sZ><sZ>10 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Sutton</wicri:noRegion></affiliation></author><author><name sortKey="Schumacher, Valerie" sort="Schumacher, Valerie" uniqKey="Schumacher V" first="Valerie" last="Schumacher">Valerie Schumacher</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Institute of Human Genetics, Heinrich-Heine University</s1><s2>Düsseldorf</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>17 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Düsseldorf</region><settlement type="city">Düsseldorf</settlement></placeName></affiliation></author><author><name sortKey="Menko, Fred H" sort="Menko, Fred H" uniqKey="Menko F" first="Fred H." last="Menko">Fred H. Menko</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</s1><s3>NLD</s3><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Pays-Bas</country><wicri:noRegion>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</wicri:noRegion></affiliation></author><author><name sortKey="Olschwang, Sylviane" sort="Olschwang, Sylviane" uniqKey="Olschwang S" first="Sylviane" last="Olschwang">Sylviane Olschwang</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Institut Paoli-Calmettes, Institut National de la Sante et de la Recherche Medicale UMR 599</s1><s2>Marseilles</s2><s3>FRA</s3><sZ>4 aut.</sZ></inist:fA14><country>France</country><wicri:noRegion>Marseilles</wicri:noRegion><wicri:noRegion>Institut National de la Sante et de la Recherche Medicale UMR 599</wicri:noRegion><wicri:noRegion>Marseilles</wicri:noRegion></affiliation></author><author><name sortKey="Boardman, Lisa A" sort="Boardman, Lisa A" uniqKey="Boardman L" first="Lisa A." last="Boardman">Lisa A. Boardman</name><affiliation wicri:level="2"><inist:fA14 i1="05"><s1>Division of Gastroenterology, Department of Internal Medicine, Mayo Clinic, Mayo Foundation</s1><s2>Rochester, Minnesota</s2><s3>USA</s3><sZ>5 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Minnesota</region></placeName></affiliation></author><author><name sortKey="Gille, Johan J P" sort="Gille, Johan J P" uniqKey="Gille J" first="Johan J. P." last="Gille">Johan J. P. Gille</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</s1><s3>NLD</s3><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Pays-Bas</country><wicri:noRegion>Department of Clinical Genetics and Human Genetics, VU University Medical Centre</wicri:noRegion></affiliation></author><author><name sortKey="Keller, Josbert J" sort="Keller, Josbert J" uniqKey="Keller J" first="Josbert J." last="Keller">Josbert J. Keller</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Pathology, Academic Medical Centre</s1><s2>Amsterdam</s2><s3>NLD</s3><sZ>7 aut.</sZ><sZ>14 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region></placeName></affiliation></author><author><name sortKey="Westerman, Anne Marie" sort="Westerman, Anne Marie" uniqKey="Westerman A" first="Anne Marie" last="Westerman">Anne Marie Westerman</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Scott, Rodney J" sort="Scott, Rodney J" uniqKey="Scott R" first="Rodney J." last="Scott">Rodney J. Scott</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Discipline of Medical Genetics, Faculty of Health, Newcastle and Hunter Medical Research Institute</s1><s2>Newcastle, New South Wales</s2><s3>AUS</s3><sZ>9 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Newcastle, New South Wales</wicri:noRegion></affiliation></author><author><name sortKey="Lim, Wendy" sort="Lim, Wendy" uniqKey="Lim W" first="Wendy" last="Lim">Wendy Lim</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Section of Cancer Genetics, Institute of Cancer Research</s1><s2>Sutton</s2><s3>GBR</s3><sZ>1 aut.</sZ><sZ>10 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Sutton</wicri:noRegion></affiliation></author><author><name sortKey="Trimbath, Jill D" sort="Trimbath, Jill D" uniqKey="Trimbath J" first="Jill D." last="Trimbath">Jill D. Trimbath</name><affiliation wicri:level="2"><inist:fA14 i1="09"><s1>Department of Medicine, Johns Hopkins University School of Medicine</s1><s2>Baltimore, Maryland</s2><s3>USA</s3><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Giardiello, Francis M" sort="Giardiello, Francis M" uniqKey="Giardiello F" first="Francis M." last="Giardiello">Francis M. Giardiello</name><affiliation wicri:level="2"><inist:fA14 i1="09"><s1>Department of Medicine, Johns Hopkins University School of Medicine</s1><s2>Baltimore, Maryland</s2><s3>USA</s3><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Gruber, Stephen B" sort="Gruber, Stephen B" uniqKey="Gruber S" first="Stephen B." last="Gruber">Stephen B. Gruber</name><affiliation wicri:level="2"><inist:fA14 i1="10"><s1>Division of Molecular Medicine and Genetics, University of Michigan</s1><s2>Ann Arbor, Michigan</s2><s3>USA</s3><sZ>13 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Michigan</region></placeName></affiliation></author><author><name sortKey="Offerhaus, G Johan A" sort="Offerhaus, G Johan A" uniqKey="Offerhaus G" first="G. Johan A." last="Offerhaus">G. Johan A. Offerhaus</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Pathology, Academic Medical Centre</s1><s2>Amsterdam</s2><s3>NLD</s3><sZ>7 aut.</sZ><sZ>14 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region></placeName></affiliation></author><author><name sortKey="De Rooij, Felix W M" sort="De Rooij, Felix W M" uniqKey="De Rooij F" first="Felix W. M." last="De Rooij">Felix W. M. De Rooij</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Wilson, J H Paul" sort="Wilson, J H Paul" uniqKey="Wilson J" first="J. H. Paul" last="Wilson">J. H. Paul Wilson</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Internal Medicine, Erasmus MC University Medical Centre</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>8 aut.</sZ><sZ>15 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Hansmann, Anika" sort="Hansmann, Anika" uniqKey="Hansmann A" first="Anika" last="Hansmann">Anika Hansmann</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Institute of Human Genetics, Heinrich-Heine University</s1><s2>Düsseldorf</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>17 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Düsseldorf</region><settlement type="city">Düsseldorf</settlement></placeName></affiliation></author><author><name sortKey="Moslein, Gabriela" sort="Moslein, Gabriela" uniqKey="Moslein G" first="Gabriela" last="Möslein">Gabriela Möslein</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of General and Visceral Surgery, St. Josefs-Hospital Bochum-Linden</s1><s2>Bochum</s2><s3>DEU</s3><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Bochum</wicri:noRegion><wicri:noRegion>St. Josefs-Hospital Bochum-Linden</wicri:noRegion><wicri:noRegion>Bochum</wicri:noRegion></affiliation></author><author><name sortKey="Royer Pokora, Brigitte" sort="Royer Pokora, Brigitte" uniqKey="Royer Pokora B" first="Brigitte" last="Royer-Pokora">Brigitte Royer-Pokora</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Institute of Human Genetics, Heinrich-Heine University</s1><s2>Düsseldorf</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>17 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Düsseldorf</region><settlement type="city">Düsseldorf</settlement></placeName></affiliation></author><author><name sortKey="Tilmanvogel" sort="Tilmanvogel" uniqKey="Tilmanvogel" last="Tilmanvogel">TILMANVOGEL</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Department of General, Visceral and Thoracic Surgery, Klinken Maria Hilf</s1><s2>Mönchengladbach</s2><s3>DEU</s3><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Mönchengladbach</wicri:noRegion><wicri:noRegion>Klinken Maria Hilf</wicri:noRegion><wicri:noRegion>Mönchengladbach</wicri:noRegion></affiliation></author><author><name sortKey="Phillips, Robin K S" sort="Phillips, Robin K S" uniqKey="Phillips R" first="Robin K. S." last="Phillips">Robin K. S. Phillips</name><affiliation wicri:level="1"><inist:fA14 i1="13"><s1>Polyposis Registry, St. Mark's Hospital</s1><s2>Harrow</s2><s3>GBR</s3><sZ>21 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Harrow</wicri:noRegion></affiliation></author><author><name sortKey="Spigelman, Allan D" sort="Spigelman, Allan D" uniqKey="Spigelman A" first="Allan D." last="Spigelman">Allan D. Spigelman</name><affiliation wicri:level="1"><inist:fA14 i1="14"><s1>Professorial Surgical Unit, Faculty of Medicine, St. Vincent's Hospital Clinical School, University of New South Wales</s1><s3>AUS</s3><sZ>22 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Professorial Surgical Unit, Faculty of Medicine, St. Vincent's Hospital Clinical School, University of New South Wales</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Hunter Family Cancer Service and New South Wales and Australian Capital Territory Hereditary Cancer Registries</s1><s2>Sydney, New South Wales</s2><s3>AUS</s3><sZ>22 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Hunter Family Cancer Service and New South Wales and Australian Capital Territory Hereditary Cancer Registries</wicri:noRegion></affiliation></author><author><name sortKey="Houlston, Richard S" sort="Houlston, Richard S" uniqKey="Houlston R" first="Richard S." last="Houlston">Richard S. Houlston</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Section of Cancer Genetics, Institute of Cancer Research</s1><s2>Sutton</s2><s3>GBR</s3><sZ>1 aut.</sZ><sZ>10 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Sutton</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Clinical cancer research</title><title level="j" type="abbreviated">Clin. cancer res.</title><idno type="ISSN">1078-0432</idno><imprint><date when="2006">2006</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Clinical cancer research</title><title level="j" type="abbreviated">Clin. cancer res.</title><idno type="ISSN">1078-0432</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Frequency</term><term>Malignant tumor</term><term>Peutz Jeghers syndrome</term><term>Spectrum</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Fréquence</term><term>Spectre</term><term>Tumeur maligne</term><term>Peutz Jeghers syndrome</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background: Although an increased cancer risk in Peutz-Jeghers syndrome is established, data on the spectrum of tumors associated with the disease and the influence of germ-line STK11I LKB1 (serine/threonine kinase) mutation status are limited. Experimental Design: We analyzed the incidence of cancer in 419 individuals with Peutz-Jeghers syndrome, and 297 had documented STK11/LKB1 mutations. Results: Ninety-six cancers were found among individuals with Peutz-Jeghers syndrome. The risk for developing cancer at ages 20, 30, 40, 50, 60, and 70 years was 2%, 6%, 17%, 31%, 60%, and 85%, respectively. The most common cancers represented in this analysis were gastrointestinal in origin, gastroesophageal, small bowel, colorectal, and pancreatic, and the risk for these cancers at ages 30, 40, 50, and 60 years was 1%, 9%, 15%, and 33%, respectively. In women with Peutz-Jeghers syndrome, the risk of breast cancer was substantially increased, being 8% and 31% at ages 40 and 60 years, respectively. Kaplan-Meier analysis showed that cancer risks were similar in Peutz-Jeghers syndrome patients with identified STK11/LKB1 mutations and those with no detectable mutation (log-rank test of difference X<sup>2</sup> = 0.62; 1 df; P = 0.43). Furthermore, the type or site of STK11/LKB1 mutation did not significantly influence cancer risk. Conclusions: The results from our study provide quantitative information on the spectrum of cancers and risks of specific cancer types associated with Peutz-Jeghers syndrome.</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>Australie</li><li>France</li><li>Pays-Bas</li><li>Royaume-Uni</li><li>États-Unis</li></country><region><li>District de Düsseldorf</li><li>Hollande-Méridionale</li><li>Hollande-Septentrionale</li><li>Maryland</li><li>Michigan</li><li>Minnesota</li><li>Rhénanie-du-Nord-Westphalie</li></region><settlement><li>Amsterdam</li><li>Düsseldorf</li><li>Rotterdam</li></settlement></list><tree><country name="Royaume-Uni"><noRegion><name sortKey="Hearle, Nicholas" sort="Hearle, Nicholas" uniqKey="Hearle N" first="Nicholas" last="Hearle">Nicholas Hearle</name></noRegion><name sortKey="Houlston, Richard S" sort="Houlston, Richard S" uniqKey="Houlston R" first="Richard S." last="Houlston">Richard S. Houlston</name><name sortKey="Lim, Wendy" sort="Lim, Wendy" uniqKey="Lim W" first="Wendy" last="Lim">Wendy Lim</name><name sortKey="Phillips, Robin K S" sort="Phillips, Robin K S" uniqKey="Phillips R" first="Robin K. S." last="Phillips">Robin K. S. Phillips</name></country><country name="Allemagne"><region name="Rhénanie-du-Nord-Westphalie"><name sortKey="Schumacher, Valerie" sort="Schumacher, Valerie" uniqKey="Schumacher V" first="Valerie" last="Schumacher">Valerie Schumacher</name></region><name sortKey="Hansmann, Anika" sort="Hansmann, Anika" uniqKey="Hansmann A" first="Anika" last="Hansmann">Anika Hansmann</name><name sortKey="Moslein, Gabriela" sort="Moslein, Gabriela" uniqKey="Moslein G" first="Gabriela" last="Möslein">Gabriela Möslein</name><name sortKey="Royer Pokora, Brigitte" sort="Royer Pokora, Brigitte" uniqKey="Royer Pokora B" first="Brigitte" last="Royer-Pokora">Brigitte Royer-Pokora</name><name sortKey="Tilmanvogel" sort="Tilmanvogel" uniqKey="Tilmanvogel" last="Tilmanvogel">TILMANVOGEL</name></country><country name="Pays-Bas"><noRegion><name sortKey="Menko, Fred H" sort="Menko, Fred H" uniqKey="Menko F" first="Fred H." last="Menko">Fred H. Menko</name></noRegion><name sortKey="De Rooij, Felix W M" sort="De Rooij, Felix W M" uniqKey="De Rooij F" first="Felix W. M." last="De Rooij">Felix W. M. De Rooij</name><name sortKey="Gille, Johan J P" sort="Gille, Johan J P" uniqKey="Gille J" first="Johan J. P." last="Gille">Johan J. P. Gille</name><name sortKey="Keller, Josbert J" sort="Keller, Josbert J" uniqKey="Keller J" first="Josbert J." last="Keller">Josbert J. Keller</name><name sortKey="Offerhaus, G Johan A" sort="Offerhaus, G Johan A" uniqKey="Offerhaus G" first="G. Johan A." last="Offerhaus">G. Johan A. Offerhaus</name><name sortKey="Westerman, Anne Marie" sort="Westerman, Anne Marie" uniqKey="Westerman A" first="Anne Marie" last="Westerman">Anne Marie Westerman</name><name sortKey="Wilson, J H Paul" sort="Wilson, J H Paul" uniqKey="Wilson J" first="J. H. Paul" last="Wilson">J. H. Paul Wilson</name></country><country name="France"><noRegion><name sortKey="Olschwang, Sylviane" sort="Olschwang, Sylviane" uniqKey="Olschwang S" first="Sylviane" last="Olschwang">Sylviane Olschwang</name></noRegion></country><country name="États-Unis"><region name="Minnesota"><name sortKey="Boardman, Lisa A" sort="Boardman, Lisa A" uniqKey="Boardman L" first="Lisa A." last="Boardman">Lisa A. Boardman</name></region><name sortKey="Giardiello, Francis M" sort="Giardiello, Francis M" uniqKey="Giardiello F" first="Francis M." last="Giardiello">Francis M. Giardiello</name><name sortKey="Gruber, Stephen B" sort="Gruber, Stephen B" uniqKey="Gruber S" first="Stephen B." last="Gruber">Stephen B. Gruber</name><name sortKey="Trimbath, Jill D" sort="Trimbath, Jill D" uniqKey="Trimbath J" first="Jill D." last="Trimbath">Jill D. Trimbath</name></country><country name="Australie"><noRegion><name sortKey="Scott, Rodney J" sort="Scott, Rodney J" uniqKey="Scott R" first="Rodney J." last="Scott">Rodney J. Scott</name></noRegion><name sortKey="Spigelman, Allan D" sort="Spigelman, Allan D" uniqKey="Spigelman A" first="Allan D." last="Spigelman">Allan D. Spigelman</name><name sortKey="Spigelman, Allan D" sort="Spigelman, Allan D" uniqKey="Spigelman A" first="Allan D." last="Spigelman">Allan D. Spigelman</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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