A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer
Background Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene, CDH1 . The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively. Methods Eighteen consecutive patient...
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| Veröffentlicht in: | Annals of surgical oncology 2011-09, Vol.18 (9), p.2594-2598 |
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| creator | Chen, Yijun Kingham, Kerry Ford, James M. Rosing, James Van Dam, Jacques Jeffrey, R. Brooke Longacre, Teri A. Chun, Nicki Kurian, Allison Norton, Jeffrey A. |
| description | Background
Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene,
CDH1
. The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively.
Methods
Eighteen consecutive patients with
CDH1
mutations and positive family history were studied prospectively, including 13 without and 5 with symptoms. Proportions were compared by Fisher’s exact test, and survival by the Breslow modification of the Wilcoxon rank-sum test.
Results
Each patient underwent total gastrectomy (TG), and 17 (94%) were found to have signet ring cell adenocarcinoma. Twelve of 13 asymptomatic patients had T1, N0 cancer, and only 2/12 (16%) had it diagnosed preoperatively despite state-of-the-art screening methods. Each asymptomatic patient did well postoperatively, and no patient has recurred. For five symptomatic patients, each (100%) was found to have signet ring cell adenocarcinoma (
P
= 0.002 versus asymptomatic) by preoperative endoscopy; three (60%) had lymph node involvement and two (40%) had distant metastases at time of operation. Two-year survival was 100% for asymptomatic and 40% for symptomatic patients (
P
|
| doi_str_mv | 10.1245/s10434-011-1648-9 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_885910857</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>885910857</sourcerecordid><originalsourceid>FETCH-LOGICAL-c436t-a9775f68f27c9087061077c9e1e47583cd19444b540d54d548794b82c014ac103</originalsourceid><addsrcrecordid>eNp1kFFLwzAUhYMobk5_gC8SfPGpmtveNOnj2HQTBg6cb0Lo0lQ6tmUmrbB_b2qngiAEcrn3O-cmh5BLYLcQI7_zwDDBiAFEkKKMsiPSBx46mEo4DjVLQzNOeY-ceb9iDETC-CnpxYAxJoL1yeuQzp31O6Pr6sPQ57op9tSWdGHrfE0nua9dGNnNnpbW0dF4CtHc-uoLnhpniqrO3Z6Oq7JsvOkElaajfKuNOycnZb725uJwD8jLw_1iNI1mT5PH0XAWaUzSOsozIXiZyjIWOmNSsBSYCKUBg4LLRBeQIeKSIys4hiNFhksZawaYa2DJgNx0vjtn3xvja7WpvDbrdb41tvFKSp4Bk1wE8voPubKN24bHtRCmXMYtBB2kQzLemVLtXLUJ31TAVBu86oJXIXjVBq-yoLk6GDfLjSl-FN9JByDuAB9G2zfjfjf_7_oJo0WLTw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>885465827</pqid></control><display><type>article</type><title>A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer</title><source>MEDLINE</source><source>SpringerLink Journals - AutoHoldings</source><creator>Chen, Yijun ; Kingham, Kerry ; Ford, James M. ; Rosing, James ; Van Dam, Jacques ; Jeffrey, R. Brooke ; Longacre, Teri A. ; Chun, Nicki ; Kurian, Allison ; Norton, Jeffrey A.</creator><creatorcontrib>Chen, Yijun ; Kingham, Kerry ; Ford, James M. ; Rosing, James ; Van Dam, Jacques ; Jeffrey, R. Brooke ; Longacre, Teri A. ; Chun, Nicki ; Kurian, Allison ; Norton, Jeffrey A.</creatorcontrib><description>Background
Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene,
CDH1
. The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively.
Methods
Eighteen consecutive patients with
CDH1
mutations and positive family history were studied prospectively, including 13 without and 5 with symptoms. Proportions were compared by Fisher’s exact test, and survival by the Breslow modification of the Wilcoxon rank-sum test.
Results
Each patient underwent total gastrectomy (TG), and 17 (94%) were found to have signet ring cell adenocarcinoma. Twelve of 13 asymptomatic patients had T1, N0 cancer, and only 2/12 (16%) had it diagnosed preoperatively despite state-of-the-art screening methods. Each asymptomatic patient did well postoperatively, and no patient has recurred. For five symptomatic patients, each (100%) was found to have signet ring cell adenocarcinoma (
P
= 0.002 versus asymptomatic) by preoperative endoscopy; three (60%) had lymph node involvement and two (40%) had distant metastases at time of operation. Two-year survival was 100% for asymptomatic and 40% for symptomatic patients (
P
< 0.01).
Conclusion
The data show that asymptomatic patients with family history of HDGC and
CDH1
mutation have high probability of having signet ring cell adenocarcinoma of the stomach that is not able to be diagnosed on endoscopy; when symptoms arise, the diagnosis can be made by endoscopy, but they have metastases and decreased survival. Surveillance endoscopy is of limited value, and prophylactic gastrectomy (PG) is recommended for patients with family history of HDGC and
CDH1
mutations.</description><identifier>ISSN: 1068-9265</identifier><identifier>EISSN: 1534-4681</identifier><identifier>DOI: 10.1245/s10434-011-1648-9</identifier><identifier>PMID: 21424370</identifier><language>eng</language><publisher>New York: Springer-Verlag</publisher><subject>Adolescent ; Adult ; Aged ; Cadherins - genetics ; Carcinoma, Signet Ring Cell - genetics ; Carcinoma, Signet Ring Cell - pathology ; Carcinoma, Signet Ring Cell - surgery ; Female ; Follow-Up Studies ; Gastrectomy ; Gastrointestinal Oncology ; Genetic Predisposition to Disease ; Humans ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Mutation - genetics ; Oncology ; Pedigree ; Prognosis ; Prospective Studies ; Stomach Neoplasms - genetics ; Stomach Neoplasms - pathology ; Stomach Neoplasms - surgery ; Surgery ; Surgical Oncology ; Survival Rate ; Young Adult</subject><ispartof>Annals of surgical oncology, 2011-09, Vol.18 (9), p.2594-2598</ispartof><rights>Society of Surgical Oncology 2011</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c436t-a9775f68f27c9087061077c9e1e47583cd19444b540d54d548794b82c014ac103</citedby><cites>FETCH-LOGICAL-c436t-a9775f68f27c9087061077c9e1e47583cd19444b540d54d548794b82c014ac103</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1245/s10434-011-1648-9$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1245/s10434-011-1648-9$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,780,784,27924,27925,41488,42557,51319</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21424370$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Chen, Yijun</creatorcontrib><creatorcontrib>Kingham, Kerry</creatorcontrib><creatorcontrib>Ford, James M.</creatorcontrib><creatorcontrib>Rosing, James</creatorcontrib><creatorcontrib>Van Dam, Jacques</creatorcontrib><creatorcontrib>Jeffrey, R. Brooke</creatorcontrib><creatorcontrib>Longacre, Teri A.</creatorcontrib><creatorcontrib>Chun, Nicki</creatorcontrib><creatorcontrib>Kurian, Allison</creatorcontrib><creatorcontrib>Norton, Jeffrey A.</creatorcontrib><title>A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer</title><title>Annals of surgical oncology</title><addtitle>Ann Surg Oncol</addtitle><addtitle>Ann Surg Oncol</addtitle><description>Background
Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene,
CDH1
. The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively.
Methods
Eighteen consecutive patients with
CDH1
mutations and positive family history were studied prospectively, including 13 without and 5 with symptoms. Proportions were compared by Fisher’s exact test, and survival by the Breslow modification of the Wilcoxon rank-sum test.
Results
Each patient underwent total gastrectomy (TG), and 17 (94%) were found to have signet ring cell adenocarcinoma. Twelve of 13 asymptomatic patients had T1, N0 cancer, and only 2/12 (16%) had it diagnosed preoperatively despite state-of-the-art screening methods. Each asymptomatic patient did well postoperatively, and no patient has recurred. For five symptomatic patients, each (100%) was found to have signet ring cell adenocarcinoma (
P
= 0.002 versus asymptomatic) by preoperative endoscopy; three (60%) had lymph node involvement and two (40%) had distant metastases at time of operation. Two-year survival was 100% for asymptomatic and 40% for symptomatic patients (
P
< 0.01).
Conclusion
The data show that asymptomatic patients with family history of HDGC and
CDH1
mutation have high probability of having signet ring cell adenocarcinoma of the stomach that is not able to be diagnosed on endoscopy; when symptoms arise, the diagnosis can be made by endoscopy, but they have metastases and decreased survival. Surveillance endoscopy is of limited value, and prophylactic gastrectomy (PG) is recommended for patients with family history of HDGC and
CDH1
mutations.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Cadherins - genetics</subject><subject>Carcinoma, Signet Ring Cell - genetics</subject><subject>Carcinoma, Signet Ring Cell - pathology</subject><subject>Carcinoma, Signet Ring Cell - surgery</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Gastrectomy</subject><subject>Gastrointestinal Oncology</subject><subject>Genetic Predisposition to Disease</subject><subject>Humans</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Mutation - genetics</subject><subject>Oncology</subject><subject>Pedigree</subject><subject>Prognosis</subject><subject>Prospective Studies</subject><subject>Stomach Neoplasms - genetics</subject><subject>Stomach Neoplasms - pathology</subject><subject>Stomach Neoplasms - surgery</subject><subject>Surgery</subject><subject>Surgical Oncology</subject><subject>Survival Rate</subject><subject>Young Adult</subject><issn>1068-9265</issn><issn>1534-4681</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp1kFFLwzAUhYMobk5_gC8SfPGpmtveNOnj2HQTBg6cb0Lo0lQ6tmUmrbB_b2qngiAEcrn3O-cmh5BLYLcQI7_zwDDBiAFEkKKMsiPSBx46mEo4DjVLQzNOeY-ceb9iDETC-CnpxYAxJoL1yeuQzp31O6Pr6sPQ57op9tSWdGHrfE0nua9dGNnNnpbW0dF4CtHc-uoLnhpniqrO3Z6Oq7JsvOkElaajfKuNOycnZb725uJwD8jLw_1iNI1mT5PH0XAWaUzSOsozIXiZyjIWOmNSsBSYCKUBg4LLRBeQIeKSIys4hiNFhksZawaYa2DJgNx0vjtn3xvja7WpvDbrdb41tvFKSp4Bk1wE8voPubKN24bHtRCmXMYtBB2kQzLemVLtXLUJ31TAVBu86oJXIXjVBq-yoLk6GDfLjSl-FN9JByDuAB9G2zfjfjf_7_oJo0WLTw</recordid><startdate>20110901</startdate><enddate>20110901</enddate><creator>Chen, Yijun</creator><creator>Kingham, Kerry</creator><creator>Ford, James M.</creator><creator>Rosing, James</creator><creator>Van Dam, Jacques</creator><creator>Jeffrey, R. Brooke</creator><creator>Longacre, Teri A.</creator><creator>Chun, Nicki</creator><creator>Kurian, Allison</creator><creator>Norton, Jeffrey A.</creator><general>Springer-Verlag</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TO</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20110901</creationdate><title>A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer</title><author>Chen, Yijun ; Kingham, Kerry ; Ford, James M. ; Rosing, James ; Van Dam, Jacques ; Jeffrey, R. Brooke ; Longacre, Teri A. ; Chun, Nicki ; Kurian, Allison ; Norton, Jeffrey A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c436t-a9775f68f27c9087061077c9e1e47583cd19444b540d54d548794b82c014ac103</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Cadherins - genetics</topic><topic>Carcinoma, Signet Ring Cell - genetics</topic><topic>Carcinoma, Signet Ring Cell - pathology</topic><topic>Carcinoma, Signet Ring Cell - surgery</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Gastrectomy</topic><topic>Gastrointestinal Oncology</topic><topic>Genetic Predisposition to Disease</topic><topic>Humans</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Mutation - genetics</topic><topic>Oncology</topic><topic>Pedigree</topic><topic>Prognosis</topic><topic>Prospective Studies</topic><topic>Stomach Neoplasms - genetics</topic><topic>Stomach Neoplasms - pathology</topic><topic>Stomach Neoplasms - surgery</topic><topic>Surgery</topic><topic>Surgical Oncology</topic><topic>Survival Rate</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Chen, Yijun</creatorcontrib><creatorcontrib>Kingham, Kerry</creatorcontrib><creatorcontrib>Ford, James M.</creatorcontrib><creatorcontrib>Rosing, James</creatorcontrib><creatorcontrib>Van Dam, Jacques</creatorcontrib><creatorcontrib>Jeffrey, R. Brooke</creatorcontrib><creatorcontrib>Longacre, Teri A.</creatorcontrib><creatorcontrib>Chun, Nicki</creatorcontrib><creatorcontrib>Kurian, Allison</creatorcontrib><creatorcontrib>Norton, Jeffrey A.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Annals of surgical oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Chen, Yijun</au><au>Kingham, Kerry</au><au>Ford, James M.</au><au>Rosing, James</au><au>Van Dam, Jacques</au><au>Jeffrey, R. Brooke</au><au>Longacre, Teri A.</au><au>Chun, Nicki</au><au>Kurian, Allison</au><au>Norton, Jeffrey A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer</atitle><jtitle>Annals of surgical oncology</jtitle><stitle>Ann Surg Oncol</stitle><addtitle>Ann Surg Oncol</addtitle><date>2011-09-01</date><risdate>2011</risdate><volume>18</volume><issue>9</issue><spage>2594</spage><epage>2598</epage><pages>2594-2598</pages><issn>1068-9265</issn><eissn>1534-4681</eissn><abstract>Background
Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene,
CDH1
. The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively.
Methods
Eighteen consecutive patients with
CDH1
mutations and positive family history were studied prospectively, including 13 without and 5 with symptoms. Proportions were compared by Fisher’s exact test, and survival by the Breslow modification of the Wilcoxon rank-sum test.
Results
Each patient underwent total gastrectomy (TG), and 17 (94%) were found to have signet ring cell adenocarcinoma. Twelve of 13 asymptomatic patients had T1, N0 cancer, and only 2/12 (16%) had it diagnosed preoperatively despite state-of-the-art screening methods. Each asymptomatic patient did well postoperatively, and no patient has recurred. For five symptomatic patients, each (100%) was found to have signet ring cell adenocarcinoma (
P
= 0.002 versus asymptomatic) by preoperative endoscopy; three (60%) had lymph node involvement and two (40%) had distant metastases at time of operation. Two-year survival was 100% for asymptomatic and 40% for symptomatic patients (
P
< 0.01).
Conclusion
The data show that asymptomatic patients with family history of HDGC and
CDH1
mutation have high probability of having signet ring cell adenocarcinoma of the stomach that is not able to be diagnosed on endoscopy; when symptoms arise, the diagnosis can be made by endoscopy, but they have metastases and decreased survival. Surveillance endoscopy is of limited value, and prophylactic gastrectomy (PG) is recommended for patients with family history of HDGC and
CDH1
mutations.</abstract><cop>New York</cop><pub>Springer-Verlag</pub><pmid>21424370</pmid><doi>10.1245/s10434-011-1648-9</doi><tpages>5</tpages></addata></record> |
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| issn | 1068-9265 1534-4681 |
| language | eng |
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| subjects | Adolescent Adult Aged Cadherins - genetics Carcinoma, Signet Ring Cell - genetics Carcinoma, Signet Ring Cell - pathology Carcinoma, Signet Ring Cell - surgery Female Follow-Up Studies Gastrectomy Gastrointestinal Oncology Genetic Predisposition to Disease Humans Male Medicine Medicine & Public Health Middle Aged Mutation - genetics Oncology Pedigree Prognosis Prospective Studies Stomach Neoplasms - genetics Stomach Neoplasms - pathology Stomach Neoplasms - surgery Surgery Surgical Oncology Survival Rate Young Adult |
| title | A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer |
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