An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a...
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| Veröffentlicht in: | Internal Medicine 2018/07/15, Vol.57(14), pp.2007-2011 |
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| container_issue | 14 |
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| container_title | Internal Medicine |
| container_volume | 57 |
| creator | Miki, Masami Kawabe, Ken Igarashi, Hisato Abe, Tatsuro Ohishi, Yoshihiro Hashimoto, Risa Karashima, Takashi Yamasaki, Ichiro Inoue, Keiji Ito, Tetsuhide Ogawa, Yoshihiro |
| description | A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease. |
| doi_str_mv | 10.2169/internalmedicine.0416-17 |
| format | Article |
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She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease.</description><identifier>ISSN: 0918-2918</identifier><identifier>EISSN: 1349-7235</identifier><identifier>DOI: 10.2169/internalmedicine.0416-17</identifier><identifier>PMID: 29491307</identifier><language>eng</language><publisher>Japan: The Japanese Society of Internal Medicine</publisher><subject>Antineoplastic Agents - therapeutic use ; Asian Continental Ancestry Group ; Autopsies ; Autopsy ; Biopsy ; Carcinoma, Neuroendocrine - drug therapy ; Carcinoma, Neuroendocrine - etiology ; Carcinoma, Neuroendocrine - physiopathology ; Case Report ; everolimus ; Everolimus - therapeutic use ; Female ; Humans ; Indoles - therapeutic use ; Internal medicine ; Liver ; Liver Neoplasms - drug therapy ; Liver Neoplasms - etiology ; Liver Neoplasms - physiopathology ; Medical treatment ; Middle Aged ; Neuroendocrine tumors ; Pancreas ; Pancreatic carcinoma ; Pancreatic Neoplasms - drug therapy ; Pancreatic Neoplasms - etiology ; Pancreatic Neoplasms - physiopathology ; pancreatic neuroendocrine tumors-G3 (NET-G3) ; Pyrroles - therapeutic use ; Sunitinib ; Treatment Outcome ; Tumors ; VHL protein ; Von Hippel-Lindau disease ; von Hippel-Lindau disease (VHL) ; von Hippel-Lindau Disease - complications ; von Hippel-Lindau Disease - physiopathology ; well-differentiated neuroendocrine carcinoma (WDNEC) ; WHO G3</subject><ispartof>Internal Medicine, 2018/07/15, Vol.57(14), pp.2007-2011</ispartof><rights>2018 by The Japanese Society of Internal Medicine</rights><rights>Copyright Japan Science and Technology Agency 2018</rights><rights>Copyright © 2018 by The Japanese Society of Internal Medicine 2018</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c555t-f00d09966891892ee5085189c71e936ea5dfcf7132010ba90b06c789343b2a313</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6096011/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6096011/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,1876,27903,27904,53769,53771</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29491307$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Miki, Masami</creatorcontrib><creatorcontrib>Kawabe, Ken</creatorcontrib><creatorcontrib>Igarashi, Hisato</creatorcontrib><creatorcontrib>Abe, Tatsuro</creatorcontrib><creatorcontrib>Ohishi, Yoshihiro</creatorcontrib><creatorcontrib>Hashimoto, Risa</creatorcontrib><creatorcontrib>Karashima, Takashi</creatorcontrib><creatorcontrib>Yamasaki, Ichiro</creatorcontrib><creatorcontrib>Inoue, Keiji</creatorcontrib><creatorcontrib>Ito, Tetsuhide</creatorcontrib><creatorcontrib>Ogawa, Yoshihiro</creatorcontrib><title>An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease</title><title>Internal Medicine</title><addtitle>Intern. Med.</addtitle><description>A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease.</description><subject>Antineoplastic Agents - therapeutic use</subject><subject>Asian Continental Ancestry Group</subject><subject>Autopsies</subject><subject>Autopsy</subject><subject>Biopsy</subject><subject>Carcinoma, Neuroendocrine - drug therapy</subject><subject>Carcinoma, Neuroendocrine - etiology</subject><subject>Carcinoma, Neuroendocrine - physiopathology</subject><subject>Case Report</subject><subject>everolimus</subject><subject>Everolimus - therapeutic use</subject><subject>Female</subject><subject>Humans</subject><subject>Indoles - therapeutic use</subject><subject>Internal medicine</subject><subject>Liver</subject><subject>Liver Neoplasms - drug therapy</subject><subject>Liver Neoplasms - etiology</subject><subject>Liver Neoplasms - physiopathology</subject><subject>Medical treatment</subject><subject>Middle Aged</subject><subject>Neuroendocrine tumors</subject><subject>Pancreas</subject><subject>Pancreatic carcinoma</subject><subject>Pancreatic Neoplasms - drug therapy</subject><subject>Pancreatic Neoplasms - etiology</subject><subject>Pancreatic Neoplasms - physiopathology</subject><subject>pancreatic neuroendocrine tumors-G3 (NET-G3)</subject><subject>Pyrroles - therapeutic use</subject><subject>Sunitinib</subject><subject>Treatment Outcome</subject><subject>Tumors</subject><subject>VHL protein</subject><subject>Von Hippel-Lindau disease</subject><subject>von Hippel-Lindau disease (VHL)</subject><subject>von Hippel-Lindau Disease - complications</subject><subject>von Hippel-Lindau Disease - physiopathology</subject><subject>well-differentiated neuroendocrine carcinoma (WDNEC)</subject><subject>WHO G3</subject><issn>0918-2918</issn><issn>1349-7235</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNplkU1vEzEQhi0EoqHwF5AlLuXg4o9de31BitLSIkUthwJHy_HONo42drB3g_j3eJUQQbmMLc8z73jmRQgzesmZ1B98GCAF22-h9c4HuKQVk4SpZ2jGRKWJ4qJ-jmZUs4bwEs7Qq5w3lIpGaf4SnXFdaSaomqH9POB5u7fBQYu_Q9-T1ncdJAiDt0N5-1JSCezgHb6DMUUIbXSp9MQLm0rvuLX44u76gdyI93iec3SHup9-WONvMeBbv9tBT5Y-tHbEVz6DzfAavehsn-HN8TxHXz9dPyxuyfL-5vNiviSuruuBdJS2VGspmzKE5gA1bepyc4qBFhJs3XauU0xwyujKarqi0qlGi0qsuBVMnKOPB93duCrLcmWsZHuzS35r0y8TrTf_ZoJfm8e4N5JqSdkkcHEUSPHHCHkwW59d2ZMNEMdsOKW6lopLXdB3T9BNHCeXCsWrAighq0I1B8qlmHOC7vQZRs1krnlqrpnMNUyV0rd_D3Mq_ONmAe4PwCYP9hFOgE3Fvh7-V66VYdUUjy1OpFvbZCCI39TSwtQ</recordid><startdate>20180715</startdate><enddate>20180715</enddate><creator>Miki, Masami</creator><creator>Kawabe, Ken</creator><creator>Igarashi, Hisato</creator><creator>Abe, Tatsuro</creator><creator>Ohishi, Yoshihiro</creator><creator>Hashimoto, Risa</creator><creator>Karashima, Takashi</creator><creator>Yamasaki, Ichiro</creator><creator>Inoue, Keiji</creator><creator>Ito, Tetsuhide</creator><creator>Ogawa, Yoshihiro</creator><general>The Japanese Society of Internal Medicine</general><general>Japan Science and Technology Agency</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>7T5</scope><scope>7TK</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20180715</creationdate><title>An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease</title><author>Miki, Masami ; Kawabe, Ken ; Igarashi, Hisato ; Abe, Tatsuro ; Ohishi, Yoshihiro ; Hashimoto, Risa ; Karashima, Takashi ; Yamasaki, Ichiro ; Inoue, Keiji ; Ito, Tetsuhide ; Ogawa, Yoshihiro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c555t-f00d09966891892ee5085189c71e936ea5dfcf7132010ba90b06c789343b2a313</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Antineoplastic Agents - therapeutic use</topic><topic>Asian Continental Ancestry Group</topic><topic>Autopsies</topic><topic>Autopsy</topic><topic>Biopsy</topic><topic>Carcinoma, Neuroendocrine - drug therapy</topic><topic>Carcinoma, Neuroendocrine - etiology</topic><topic>Carcinoma, Neuroendocrine - physiopathology</topic><topic>Case Report</topic><topic>everolimus</topic><topic>Everolimus - therapeutic use</topic><topic>Female</topic><topic>Humans</topic><topic>Indoles - therapeutic use</topic><topic>Internal medicine</topic><topic>Liver</topic><topic>Liver Neoplasms - drug therapy</topic><topic>Liver Neoplasms - etiology</topic><topic>Liver Neoplasms - physiopathology</topic><topic>Medical treatment</topic><topic>Middle Aged</topic><topic>Neuroendocrine tumors</topic><topic>Pancreas</topic><topic>Pancreatic carcinoma</topic><topic>Pancreatic Neoplasms - drug therapy</topic><topic>Pancreatic Neoplasms - etiology</topic><topic>Pancreatic Neoplasms - physiopathology</topic><topic>pancreatic neuroendocrine tumors-G3 (NET-G3)</topic><topic>Pyrroles - therapeutic use</topic><topic>Sunitinib</topic><topic>Treatment Outcome</topic><topic>Tumors</topic><topic>VHL protein</topic><topic>Von Hippel-Lindau disease</topic><topic>von Hippel-Lindau disease (VHL)</topic><topic>von Hippel-Lindau Disease - complications</topic><topic>von Hippel-Lindau Disease - physiopathology</topic><topic>well-differentiated neuroendocrine carcinoma (WDNEC)</topic><topic>WHO G3</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Miki, Masami</creatorcontrib><creatorcontrib>Kawabe, Ken</creatorcontrib><creatorcontrib>Igarashi, Hisato</creatorcontrib><creatorcontrib>Abe, Tatsuro</creatorcontrib><creatorcontrib>Ohishi, Yoshihiro</creatorcontrib><creatorcontrib>Hashimoto, Risa</creatorcontrib><creatorcontrib>Karashima, Takashi</creatorcontrib><creatorcontrib>Yamasaki, Ichiro</creatorcontrib><creatorcontrib>Inoue, Keiji</creatorcontrib><creatorcontrib>Ito, Tetsuhide</creatorcontrib><creatorcontrib>Ogawa, Yoshihiro</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Miki, Masami</au><au>Kawabe, Ken</au><au>Igarashi, Hisato</au><au>Abe, Tatsuro</au><au>Ohishi, Yoshihiro</au><au>Hashimoto, Risa</au><au>Karashima, Takashi</au><au>Yamasaki, Ichiro</au><au>Inoue, Keiji</au><au>Ito, Tetsuhide</au><au>Ogawa, Yoshihiro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. Med.</addtitle><date>2018-07-15</date><risdate>2018</risdate><volume>57</volume><issue>14</issue><spage>2007</spage><epage>2011</epage><pages>2007-2011</pages><issn>0918-2918</issn><eissn>1349-7235</eissn><abstract>A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease.</abstract><cop>Japan</cop><pub>The Japanese Society of Internal Medicine</pub><pmid>29491307</pmid><doi>10.2169/internalmedicine.0416-17</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Internal Medicine, 2018/07/15, Vol.57(14), pp.2007-2011 |
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| source | MEDLINE; PubMed Central Open Access; J-STAGE (Japan Science & Technology Information Aggregator, Electronic) Freely Available Titles - Japanese; PubMed Central |
| subjects | Antineoplastic Agents - therapeutic use Asian Continental Ancestry Group Autopsies Autopsy Biopsy Carcinoma, Neuroendocrine - drug therapy Carcinoma, Neuroendocrine - etiology Carcinoma, Neuroendocrine - physiopathology Case Report everolimus Everolimus - therapeutic use Female Humans Indoles - therapeutic use Internal medicine Liver Liver Neoplasms - drug therapy Liver Neoplasms - etiology Liver Neoplasms - physiopathology Medical treatment Middle Aged Neuroendocrine tumors Pancreas Pancreatic carcinoma Pancreatic Neoplasms - drug therapy Pancreatic Neoplasms - etiology Pancreatic Neoplasms - physiopathology pancreatic neuroendocrine tumors-G3 (NET-G3) Pyrroles - therapeutic use Sunitinib Treatment Outcome Tumors VHL protein Von Hippel-Lindau disease von Hippel-Lindau disease (VHL) von Hippel-Lindau Disease - complications von Hippel-Lindau Disease - physiopathology well-differentiated neuroendocrine carcinoma (WDNEC) WHO G3 |
| title | An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease |
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