Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies
Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel‐Lindau disease (VHL), neurofibromatosis 1 (NF‐1) (von Recklinghausen disease), and the tuberous sclerosis complex (TSC). The relative frequency with which p...
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| description | Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel‐Lindau disease (VHL), neurofibromatosis 1 (NF‐1) (von Recklinghausen disease), and the tuberous sclerosis complex (TSC). The relative frequency with which patients who have these disorders develop PETs is MEN1>VHL>NF‐1>TSC. Over the last few years, there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization and the medical and surgical treatment of PETs in such patients. The study of PETs in these disorders not only has provided insights into the possible pathogenesis of sporadic PETs but also has presented several unique management and treatment issues, some of which are applicable to patients with sporadic PETs. Therefore, the study of PETs in these uncommon disorders has provided valuable insights that, in many cases, are applicable to the general group of patients with sporadic PETs. In this article, these areas are reviewed briefly along with the current state of knowledge of the PETs in these disorders, and the controversies that exist in their management are summarized briefly and discussed. Cancer 2008;113(7 suppl):1807–43. Published 2008 American Cancer Society. |
| doi_str_mv | 10.1002/cncr.23648 |
| format | Article |
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The relative frequency with which patients who have these disorders develop PETs is MEN1>VHL>NF‐1>TSC. Over the last few years, there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization and the medical and surgical treatment of PETs in such patients. The study of PETs in these disorders not only has provided insights into the possible pathogenesis of sporadic PETs but also has presented several unique management and treatment issues, some of which are applicable to patients with sporadic PETs. Therefore, the study of PETs in these uncommon disorders has provided valuable insights that, in many cases, are applicable to the general group of patients with sporadic PETs. In this article, these areas are reviewed briefly along with the current state of knowledge of the PETs in these disorders, and the controversies that exist in their management are summarized briefly and discussed. Cancer 2008;113(7 suppl):1807–43. Published 2008 American Cancer Society.</description><identifier>ISSN: 0008-543X</identifier><identifier>EISSN: 1097-0142</identifier><identifier>DOI: 10.1002/cncr.23648</identifier><identifier>PMID: 18798544</identifier><identifier>CODEN: CANCAR</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Biological and medical sciences ; gastrinomas ; Gastroenterology. Liver. Pancreas. Abdomen ; Humans ; insulinomas ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Medical sciences ; Multiple Endocrine Neoplasia type 1 ; Multiple Endocrine Neoplasia Type 1 - diagnosis ; Multiple Endocrine Neoplasia Type 1 - genetics ; Multiple Endocrine Neoplasia Type 1 - therapy ; neuroendocrine tumors ; neurofibromatosis 1 ; Neurofibromatosis 1 - diagnosis ; Neurofibromatosis 1 - genetics ; Neurofibromatosis 1 - therapy ; pancreatic endocrine tumors ; Pancreatic Neoplasms - diagnosis ; Pancreatic Neoplasms - genetics ; Pancreatic Neoplasms - therapy ; Prognosis ; tuberous sclerosis ; Tuberous Sclerosis - diagnosis ; Tuberous Sclerosis - genetics ; Tuberous Sclerosis - therapy ; Tumors ; von Hippel-Lindau Disease - diagnosis ; von Hippel-Lindau Disease - genetics ; von Hippel-Lindau Disease - therapy ; von Hippel‐Lindau disease ; Zollinger‐Ellison syndrome</subject><ispartof>Cancer, 2008-10, Vol.113 (S7), p.1807-1843</ispartof><rights>Copyright © 2008 American Cancer Society</rights><rights>2008 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5148-a6623cf7141d1b773b669e8ef59a5a11e6b9bf8751630d5ea681817ba1bf68313</citedby><cites>FETCH-LOGICAL-c5148-a6623cf7141d1b773b669e8ef59a5a11e6b9bf8751630d5ea681817ba1bf68313</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fcncr.23648$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fcncr.23648$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>230,314,776,780,881,1411,1427,27901,27902,45550,45551,46384,46808</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=20702512$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18798544$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Jensen, Robert T.</creatorcontrib><creatorcontrib>Berna, Marc J.</creatorcontrib><creatorcontrib>Bingham, David B.</creatorcontrib><creatorcontrib>Norton, Jeffrey A.</creatorcontrib><title>Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies</title><title>Cancer</title><addtitle>Cancer</addtitle><description>Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel‐Lindau disease (VHL), neurofibromatosis 1 (NF‐1) (von Recklinghausen disease), and the tuberous sclerosis complex (TSC). The relative frequency with which patients who have these disorders develop PETs is MEN1>VHL>NF‐1>TSC. Over the last few years, there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization and the medical and surgical treatment of PETs in such patients. The study of PETs in these disorders not only has provided insights into the possible pathogenesis of sporadic PETs but also has presented several unique management and treatment issues, some of which are applicable to patients with sporadic PETs. Therefore, the study of PETs in these uncommon disorders has provided valuable insights that, in many cases, are applicable to the general group of patients with sporadic PETs. In this article, these areas are reviewed briefly along with the current state of knowledge of the PETs in these disorders, and the controversies that exist in their management are summarized briefly and discussed. Cancer 2008;113(7 suppl):1807–43. Published 2008 American Cancer Society.</description><subject>Biological and medical sciences</subject><subject>gastrinomas</subject><subject>Gastroenterology. Liver. Pancreas. Abdomen</subject><subject>Humans</subject><subject>insulinomas</subject><subject>Liver. Biliary tract. Portal circulation. Exocrine pancreas</subject><subject>Medical sciences</subject><subject>Multiple Endocrine Neoplasia type 1</subject><subject>Multiple Endocrine Neoplasia Type 1 - diagnosis</subject><subject>Multiple Endocrine Neoplasia Type 1 - genetics</subject><subject>Multiple Endocrine Neoplasia Type 1 - therapy</subject><subject>neuroendocrine tumors</subject><subject>neurofibromatosis 1</subject><subject>Neurofibromatosis 1 - diagnosis</subject><subject>Neurofibromatosis 1 - genetics</subject><subject>Neurofibromatosis 1 - therapy</subject><subject>pancreatic endocrine tumors</subject><subject>Pancreatic Neoplasms - diagnosis</subject><subject>Pancreatic Neoplasms - genetics</subject><subject>Pancreatic Neoplasms - therapy</subject><subject>Prognosis</subject><subject>tuberous sclerosis</subject><subject>Tuberous Sclerosis - diagnosis</subject><subject>Tuberous Sclerosis - genetics</subject><subject>Tuberous Sclerosis - therapy</subject><subject>Tumors</subject><subject>von Hippel-Lindau Disease - diagnosis</subject><subject>von Hippel-Lindau Disease - genetics</subject><subject>von Hippel-Lindau Disease - therapy</subject><subject>von Hippel‐Lindau disease</subject><subject>Zollinger‐Ellison syndrome</subject><issn>0008-543X</issn><issn>1097-0142</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kU9v1DAQxS0EokvhwgdAvsABNcVO4j_hgFStaKlUgYRA4mZNnMmuUWIvdrJoJT487u6qtBdOHmt-895oHiEvOTvnjJXvrLfxvKxkrR-RBWeNKhivy8dkwRjThairHyfkWUo_81eVonpKTrhWjRZ1vSB_rv0ao5uwoxvIOgiTsxR9F2x0Huk0jyHStPNdDCOm9_Si22YOE3WejmFAOw8Q8-y0Div0mFw6o52DlQ_7cgQPKxzRT2cUfEdt8FMMW4zJYXpOnvQwJHxxfE_J98uP35afipsvV9fLi5vCCl7rAqQsK9srXvOOt0pVrZQNauxFAwI4R9k2ba-V4LJinUCQmmuuWuBtL3XFq1Py4aC7mdsRO5u3iTCYTXQjxJ0J4MzDjndrswpbUwpV56tlgTdHgRh-zZgmM7pkcRjAY5iTkY1omNyDbw-gjSGliP2dCWfmNixzG5bZh5XhV_fX-oce08nA6yMAycLQx3x5l-64kilWCl5mjh-4327A3X8szfLz8uvB_C_tZLAt</recordid><startdate>20081001</startdate><enddate>20081001</enddate><creator>Jensen, Robert T.</creator><creator>Berna, Marc J.</creator><creator>Bingham, David B.</creator><creator>Norton, Jeffrey A.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley-Liss</general><scope>IQODW</scope><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>7X8</scope><scope>5PM</scope></search><sort><creationdate>20081001</creationdate><title>Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies</title><author>Jensen, Robert T. ; Berna, Marc J. ; Bingham, David B. ; Norton, Jeffrey A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5148-a6623cf7141d1b773b669e8ef59a5a11e6b9bf8751630d5ea681817ba1bf68313</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Biological and medical sciences</topic><topic>gastrinomas</topic><topic>Gastroenterology. Liver. Pancreas. Abdomen</topic><topic>Humans</topic><topic>insulinomas</topic><topic>Liver. Biliary tract. Portal circulation. Exocrine pancreas</topic><topic>Medical sciences</topic><topic>Multiple Endocrine Neoplasia type 1</topic><topic>Multiple Endocrine Neoplasia Type 1 - diagnosis</topic><topic>Multiple Endocrine Neoplasia Type 1 - genetics</topic><topic>Multiple Endocrine Neoplasia Type 1 - therapy</topic><topic>neuroendocrine tumors</topic><topic>neurofibromatosis 1</topic><topic>Neurofibromatosis 1 - diagnosis</topic><topic>Neurofibromatosis 1 - genetics</topic><topic>Neurofibromatosis 1 - therapy</topic><topic>pancreatic endocrine tumors</topic><topic>Pancreatic Neoplasms - diagnosis</topic><topic>Pancreatic Neoplasms - genetics</topic><topic>Pancreatic Neoplasms - therapy</topic><topic>Prognosis</topic><topic>tuberous sclerosis</topic><topic>Tuberous Sclerosis - diagnosis</topic><topic>Tuberous Sclerosis - genetics</topic><topic>Tuberous Sclerosis - therapy</topic><topic>Tumors</topic><topic>von Hippel-Lindau Disease - diagnosis</topic><topic>von Hippel-Lindau Disease - genetics</topic><topic>von Hippel-Lindau Disease - therapy</topic><topic>von Hippel‐Lindau disease</topic><topic>Zollinger‐Ellison syndrome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Jensen, Robert T.</creatorcontrib><creatorcontrib>Berna, Marc J.</creatorcontrib><creatorcontrib>Bingham, David B.</creatorcontrib><creatorcontrib>Norton, Jeffrey A.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Jensen, Robert T.</au><au>Berna, Marc J.</au><au>Bingham, David B.</au><au>Norton, Jeffrey A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies</atitle><jtitle>Cancer</jtitle><addtitle>Cancer</addtitle><date>2008-10-01</date><risdate>2008</risdate><volume>113</volume><issue>S7</issue><spage>1807</spage><epage>1843</epage><pages>1807-1843</pages><issn>0008-543X</issn><eissn>1097-0142</eissn><coden>CANCAR</coden><abstract>Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel‐Lindau disease (VHL), neurofibromatosis 1 (NF‐1) (von Recklinghausen disease), and the tuberous sclerosis complex (TSC). The relative frequency with which patients who have these disorders develop PETs is MEN1>VHL>NF‐1>TSC. Over the last few years, there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization and the medical and surgical treatment of PETs in such patients. The study of PETs in these disorders not only has provided insights into the possible pathogenesis of sporadic PETs but also has presented several unique management and treatment issues, some of which are applicable to patients with sporadic PETs. Therefore, the study of PETs in these uncommon disorders has provided valuable insights that, in many cases, are applicable to the general group of patients with sporadic PETs. In this article, these areas are reviewed briefly along with the current state of knowledge of the PETs in these disorders, and the controversies that exist in their management are summarized briefly and discussed. Cancer 2008;113(7 suppl):1807–43. Published 2008 American Cancer Society.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>18798544</pmid><doi>10.1002/cncr.23648</doi><tpages>37</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Biological and medical sciences gastrinomas Gastroenterology. Liver. Pancreas. Abdomen Humans insulinomas Liver. Biliary tract. Portal circulation. Exocrine pancreas Medical sciences Multiple Endocrine Neoplasia type 1 Multiple Endocrine Neoplasia Type 1 - diagnosis Multiple Endocrine Neoplasia Type 1 - genetics Multiple Endocrine Neoplasia Type 1 - therapy neuroendocrine tumors neurofibromatosis 1 Neurofibromatosis 1 - diagnosis Neurofibromatosis 1 - genetics Neurofibromatosis 1 - therapy pancreatic endocrine tumors Pancreatic Neoplasms - diagnosis Pancreatic Neoplasms - genetics Pancreatic Neoplasms - therapy Prognosis tuberous sclerosis Tuberous Sclerosis - diagnosis Tuberous Sclerosis - genetics Tuberous Sclerosis - therapy Tumors von Hippel-Lindau Disease - diagnosis von Hippel-Lindau Disease - genetics von Hippel-Lindau Disease - therapy von Hippel‐Lindau disease Zollinger‐Ellison syndrome |
| title | Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies |
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