Frequency and spectrum of cancers in the peutz-jeghers syndrome
Identifieur interne : 001E18 ( PascalFrancis/Curation ); précédent : 001E17; suivant : 001E19Frequency 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.
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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.
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Trimbath</name><affiliation wicri:level="1"><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></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="1"><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></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="1"><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></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="1"><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></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="1"><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></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="1"><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></affiliation></author><author><name sortKey="Hansmann, Anika" sort="Hansmann, Anika" uniqKey="Hansmann A" first="Anika" last="Hansmann">Anika Hansmann</name><affiliation wicri:level="1"><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></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></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="1"><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></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></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></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></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></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></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></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></affiliation></author><author><name sortKey="Schumacher, Valerie" sort="Schumacher, Valerie" uniqKey="Schumacher V" first="Valerie" last="Schumacher">Valerie Schumacher</name><affiliation wicri:level="1"><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></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></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></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="1"><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></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></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="1"><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></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="1"><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></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></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></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="1"><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></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="1"><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></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="1"><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></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="1"><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></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="1"><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></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="1"><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></affiliation></author><author><name sortKey="Hansmann, Anika" sort="Hansmann, Anika" uniqKey="Hansmann A" first="Anika" last="Hansmann">Anika Hansmann</name><affiliation wicri:level="1"><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></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></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="1"><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></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></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></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></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></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></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><inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>1078-0432</s0></fA01><fA03 i2="1"><s0>Clin. cancer res.</s0></fA03><fA05><s2>12</s2></fA05><fA06><s2>10</s2></fA06><fA08 i1="01" i2="1" l="ENG"><s1>Frequency and spectrum of cancers in the peutz-jeghers syndrome</s1></fA08><fA11 i1="01" i2="1"><s1>HEARLE (Nicholas)</s1></fA11><fA11 i1="02" i2="1"><s1>SCHUMACHER (Valerie)</s1></fA11><fA11 i1="03" i2="1"><s1>MENKO (Fred H.)</s1></fA11><fA11 i1="04" i2="1"><s1>OLSCHWANG (Sylviane)</s1></fA11><fA11 i1="05" i2="1"><s1>BOARDMAN (Lisa A.)</s1></fA11><fA11 i1="06" i2="1"><s1>GILLE (Johan J. P.)</s1></fA11><fA11 i1="07" i2="1"><s1>KELLER (Josbert J.)</s1></fA11><fA11 i1="08" i2="1"><s1>WESTERMAN (Anne Marie)</s1></fA11><fA11 i1="09" i2="1"><s1>SCOTT (Rodney J.)</s1></fA11><fA11 i1="10" i2="1"><s1>LIM (Wendy)</s1></fA11><fA11 i1="11" i2="1"><s1>TRIMBATH (Jill D.)</s1></fA11><fA11 i1="12" i2="1"><s1>GIARDIELLO (Francis M.)</s1></fA11><fA11 i1="13" i2="1"><s1>GRUBER (Stephen B.)</s1></fA11><fA11 i1="14" i2="1"><s1>OFFERHAUS (G. Johan A.)</s1></fA11><fA11 i1="15" i2="1"><s1>DE ROOIJ (Felix W. M.)</s1></fA11><fA11 i1="16" i2="1"><s1>WILSON (J. H. Paul)</s1></fA11><fA11 i1="17" i2="1"><s1>HANSMANN (Anika)</s1></fA11><fA11 i1="18" i2="1"><s1>MÖSLEIN (Gabriela)</s1></fA11><fA11 i1="19" i2="1"><s1>ROYER-POKORA (Brigitte)</s1></fA11><fA11 i1="20" i2="1"><s1>TILMANVOGEL</s1></fA11><fA11 i1="21" i2="1"><s1>PHILLIPS (Robin K. S.)</s1></fA11><fA11 i1="22" i2="1"><s1>SPIGELMAN (Allan D.)</s1></fA11><fA11 i1="23" i2="1"><s1>HOULSTON (Richard S.)</s1></fA11><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><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></fA14><fA14 i1="13"><s1>Polyposis Registry, St. Mark's Hospital</s1><s2>Harrow</s2><s3>GBR</s3><sZ>21 aut.</sZ></fA14><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></fA14><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></fA14><fA20><s1>3209-3215</s1></fA20><fA21><s1>2006</s1></fA21><fA23 i1="01"><s0>ENG</s0></fA23><fA43 i1="01"><s1>INIST</s1><s2>26073</s2><s5>354000115497640350</s5></fA43><fA44><s0>0000</s0><s1>© 2006 INIST-CNRS. All rights reserved.</s1></fA44><fA45><s0>43 ref.</s0></fA45><fA47 i1="01" i2="1"><s0>06-0368710</s0></fA47><fA60><s1>P</s1></fA60><fA61><s0>A</s0></fA61><fA64 i1="01" i2="1"><s0>Clinical cancer research</s0></fA64><fA66 i1="01"><s0>USA</s0></fA66><fC01 i1="01" l="ENG"><s0>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.</s0></fC01><fC02 i1="01" i2="X"><s0>002B02R</s0></fC02><fC02 i1="02" i2="X"><s0>002B08F</s0></fC02><fC03 i1="01" i2="X" l="FRE"><s0>Fréquence</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="ENG"><s0>Frequency</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="SPA"><s0>Frecuencia</s0><s5>01</s5></fC03><fC03 i1="02" i2="X" l="FRE"><s0>Spectre</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="ENG"><s0>Spectrum</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="SPA"><s0>Espectro</s0><s5>02</s5></fC03><fC03 i1="03" i2="X" l="FRE"><s0>Tumeur maligne</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" l="ENG"><s0>Malignant tumor</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" l="SPA"><s0>Tumor maligno</s0><s5>03</s5></fC03><fC03 i1="04" i2="X" l="FRE"><s0>Peutz Jeghers syndrome</s0><s5>04</s5></fC03><fC03 i1="04" i2="X" l="ENG"><s0>Peutz Jeghers syndrome</s0><s5>04</s5></fC03><fC03 i1="04" i2="X" l="SPA"><s0>Peutz Jeghers síndrome</s0><s5>04</s5></fC03><fC07 i1="01" i2="X" l="FRE"><s0>Appareil digestif pathologie</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="ENG"><s0>Digestive diseases</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="SPA"><s0>Aparato digestivo patología</s0><s5>37</s5></fC07><fC07 i1="02" i2="X" l="FRE"><s0>Maladie héréditaire</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="ENG"><s0>Genetic disease</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0><s5>38</s5></fC07><fC07 i1="03" i2="X" l="FRE"><s0>Peau pathologie</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="ENG"><s0>Skin disease</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="SPA"><s0>Piel patología</s0><s5>39</s5></fC07><fC07 i1="04" i2="X" l="FRE"><s0>Tumeur bénigne</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="ENG"><s0>Benign neoplasm</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="SPA"><s0>Tumor benigno</s0><s5>40</s5></fC07><fC07 i1="05" i2="X" l="FRE"><s0>Lentiginose</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="ENG"><s0>Lentiginosis</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="SPA"><s0>Lentiginosis</s0><s5>41</s5></fC07><fC07 i1="06" i2="X" l="FRE"><s0>Polypose</s0><s5>42</s5></fC07><fC07 i1="06" i2="X" l="ENG"><s0>Polyposis</s0><s5>42</s5></fC07><fC07 i1="06" i2="X" l="SPA"><s0>Poliposis</s0><s5>42</s5></fC07><fC07 i1="07" i2="X" l="FRE"><s0>Trouble pigmentation</s0><s5>43</s5></fC07><fC07 i1="07" i2="X" l="ENG"><s0>Pigmentation disorder</s0><s5>43</s5></fC07><fC07 i1="07" i2="X" l="SPA"><s0>Trastorno pigmentación</s0><s5>43</s5></fC07><fN21><s1>247</s1></fN21><fN44 i1="01"><s1>OTO</s1></fN44><fN82><s1>OTO</s1></fN82></pA></standard></inist></record>Pour manipuler ce document sous Unix (Dilib)
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