Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort
Background & Aims Accumulating data has shown the rising incidence and poor prognosis of early‐onset gastrointestinal cancers, but few data exist on biliary tract cancers (BTC). We aimed to analyse the clinico‐pathological, molecular, therapeutic characteristics and prognosis of patients with ea...
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| Veröffentlicht in: | Liver international 2024-08, Vol.44 (8), p.1886-1899 |
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| creator | Lebeaud, Antoine Antoun, Leony Paccard, Jane‐Rose Edeline, Julien Bourien, Hélène Fares, Nadim Tournigand, Christophe Lecomte, Thierry Tougeron, David Hautefeuille, Vincent Viénot, Angélique Henriques, Julie Williet, Nicolas Bachet, Jean‐Baptiste Smolenschi, Cristina Hollebecque, Antoine Macarulla, Teresa Castet, Florian Malka, David Neuzillet, Cindy Vernerey, Dewi Boilève, Alice Turpin, Anthony |
| description | Background & Aims
Accumulating data has shown the rising incidence and poor prognosis of early‐onset gastrointestinal cancers, but few data exist on biliary tract cancers (BTC). We aimed to analyse the clinico‐pathological, molecular, therapeutic characteristics and prognosis of patients with early onset BTC (EOBTC, age ≤50 years at diagnosis), versus olders.
Methods
We analysed patients diagnosed with intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and gallbladder adenocarcinoma between 1 January 2003 and 30 June 2021. Baseline characteristics and treatment were described in each group and compared. Progression‐free survival, overall survival and disease‐free survival were estimated in each group using the Kaplan‐Meier method.
Results
Overall, 1256 patients were included, 188 (15%) with EOBTC. Patients with EOBTC demonstrated fewer comorbidities (63.5% vs. 84.5%, p |
| doi_str_mv | 10.1111/liv.15922 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_3035074210</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>3081087523</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3482-8657f9d74be5b45d7d47134e302facf34225ccd15c311a443899d2c1a9de31133</originalsourceid><addsrcrecordid>eNp1kctOGzEUhq2KilDKgheoLLGBRcBXxtNdEgWIFJoqarsdOfaZYjSXYHuCsuueDc_Ik-A0kEWlemPr6POnc86P0DEl5zSdi8qtzqnMGfuADqjIVJ8zTvd2b8Z76FMI94TQPJd0H_W4kkoRTg_Q061u9G-ooYm4LfHCVU77NY5em4iNbgz4gF2DQftq_fLnuW0CRLzU0aUf4Sse4AZCBIvrrorOpCJ47CH6NizBRLcCHGJn1xt5vAM8GA2GE3w9no9mc_x9Pvs2G06m2LR3rY-f0cdSVwGO3u5D9PNq_GN005_OriejwbRvuFCsry5lVuY2EwuQCyFtZkVGuQBOWKlNyQVj0hhLpeGUaiG4ynPLDNW5hVTh_BCdbr1L3z50qf2idsFAVekG2i4UnHBJMsEoSejJP-h92_kmdZcoRYnKJNsIz7aUSWMHD2Wx9K5OeywoKTYJFSmh4m9Cif3yZuwWNdgd-R5JAi62wKOrYP1_UzGd_NoqXwHW35qK</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>3081087523</pqid></control><display><type>article</type><title>Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Lebeaud, Antoine ; Antoun, Leony ; Paccard, Jane‐Rose ; Edeline, Julien ; Bourien, Hélène ; Fares, Nadim ; Tournigand, Christophe ; Lecomte, Thierry ; Tougeron, David ; Hautefeuille, Vincent ; Viénot, Angélique ; Henriques, Julie ; Williet, Nicolas ; Bachet, Jean‐Baptiste ; Smolenschi, Cristina ; Hollebecque, Antoine ; Macarulla, Teresa ; Castet, Florian ; Malka, David ; Neuzillet, Cindy ; Vernerey, Dewi ; Boilève, Alice ; Turpin, Anthony</creator><creatorcontrib>Lebeaud, Antoine ; Antoun, Leony ; Paccard, Jane‐Rose ; Edeline, Julien ; Bourien, Hélène ; Fares, Nadim ; Tournigand, Christophe ; Lecomte, Thierry ; Tougeron, David ; Hautefeuille, Vincent ; Viénot, Angélique ; Henriques, Julie ; Williet, Nicolas ; Bachet, Jean‐Baptiste ; Smolenschi, Cristina ; Hollebecque, Antoine ; Macarulla, Teresa ; Castet, Florian ; Malka, David ; Neuzillet, Cindy ; Vernerey, Dewi ; Boilève, Alice ; Turpin, Anthony</creatorcontrib><description>Background & Aims
Accumulating data has shown the rising incidence and poor prognosis of early‐onset gastrointestinal cancers, but few data exist on biliary tract cancers (BTC). We aimed to analyse the clinico‐pathological, molecular, therapeutic characteristics and prognosis of patients with early onset BTC (EOBTC, age ≤50 years at diagnosis), versus olders.
Methods
We analysed patients diagnosed with intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and gallbladder adenocarcinoma between 1 January 2003 and 30 June 2021. Baseline characteristics and treatment were described in each group and compared. Progression‐free survival, overall survival and disease‐free survival were estimated in each group using the Kaplan‐Meier method.
Results
Overall, 1256 patients were included, 188 (15%) with EOBTC. Patients with EOBTC demonstrated fewer comorbidities (63.5% vs. 84.5%, p < .0001), higher tumour stage (cT3–4: 50.0% vs. 32.3%, p = .0162), bilobar liver involvement (47.8% vs. 32.1%, p = .0002), and metastatic disease (67.6% vs. 57.5%, p = .0097) compared to older. Patients with EOBTC received second‐line therapy more frequently (89.5% vs. 81.0% non‐EOBTC, p = .0224). For unresectable patients with BTC, median overall survival was 17.0 vs. 16.2 months (p = .0876), and median progression‐free survival was 5.8 vs. 6.0 months (p = .8293), in EOBTC vs. older. In advanced stages, fewer actionable alterations were found in EOBTC (e.g., IDH1 mutations [7.8% vs. 16.6%]; FGFR2‐fusion [11.7% vs. 8.9%]; p = .029).
Conclusions
Patients with EOBTC have a more advanced disease at diagnosis, are treated more heavily at an advanced stage but show similar survival. A distinctive molecular profile enriched for FGRF2 fusions was found.</description><identifier>ISSN: 1478-3223</identifier><identifier>ISSN: 1478-3231</identifier><identifier>EISSN: 1478-3231</identifier><identifier>DOI: 10.1111/liv.15922</identifier><identifier>PMID: 38588031</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Adenocarcinoma ; Adenocarcinoma - mortality ; Adenocarcinoma - pathology ; Adenocarcinoma - therapy ; Adult ; Age of Onset ; Aged ; Bile Duct Neoplasms - mortality ; Bile Duct Neoplasms - pathology ; Bile Duct Neoplasms - therapy ; Biliary tract ; biliary tract cancers ; Biliary tract diseases ; Biliary Tract Neoplasms - mortality ; Biliary Tract Neoplasms - pathology ; Biliary Tract Neoplasms - therapy ; Cancer ; chemotherapy ; Cholangiocarcinoma ; Cholangiocarcinoma - mortality ; Cholangiocarcinoma - pathology ; Cholangiocarcinoma - therapy ; Comorbidity ; Diagnosis ; early onset ; Female ; Fibroblast growth factor receptor 2 ; Gallbladder ; Gallbladder cancer ; Gallbladder Neoplasms - mortality ; Gallbladder Neoplasms - pathology ; Gallbladder Neoplasms - therapy ; Humans ; Kaplan-Meier Estimate ; Male ; Medical prognosis ; Metastases ; Middle Aged ; molecular testing ; Prognosis ; Progression-Free Survival ; Retrospective Studies ; Survival</subject><ispartof>Liver international, 2024-08, Vol.44 (8), p.1886-1899</ispartof><rights>2024 The Authors. published by John Wiley & Sons Ltd.</rights><rights>2024 The Authors. Liver International published by John Wiley & Sons Ltd.</rights><rights>2024. This article is published under http://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3482-8657f9d74be5b45d7d47134e302facf34225ccd15c311a443899d2c1a9de31133</cites><orcidid>0000-0002-2282-0101 ; 0000-0002-8289-7741 ; 0000-0002-5416-5383 ; 0000-0001-8979-3972</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fliv.15922$$EPDF$$P50$$Gwiley$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fliv.15922$$EHTML$$P50$$Gwiley$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38588031$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Lebeaud, Antoine</creatorcontrib><creatorcontrib>Antoun, Leony</creatorcontrib><creatorcontrib>Paccard, Jane‐Rose</creatorcontrib><creatorcontrib>Edeline, Julien</creatorcontrib><creatorcontrib>Bourien, Hélène</creatorcontrib><creatorcontrib>Fares, Nadim</creatorcontrib><creatorcontrib>Tournigand, Christophe</creatorcontrib><creatorcontrib>Lecomte, Thierry</creatorcontrib><creatorcontrib>Tougeron, David</creatorcontrib><creatorcontrib>Hautefeuille, Vincent</creatorcontrib><creatorcontrib>Viénot, Angélique</creatorcontrib><creatorcontrib>Henriques, Julie</creatorcontrib><creatorcontrib>Williet, Nicolas</creatorcontrib><creatorcontrib>Bachet, Jean‐Baptiste</creatorcontrib><creatorcontrib>Smolenschi, Cristina</creatorcontrib><creatorcontrib>Hollebecque, Antoine</creatorcontrib><creatorcontrib>Macarulla, Teresa</creatorcontrib><creatorcontrib>Castet, Florian</creatorcontrib><creatorcontrib>Malka, David</creatorcontrib><creatorcontrib>Neuzillet, Cindy</creatorcontrib><creatorcontrib>Vernerey, Dewi</creatorcontrib><creatorcontrib>Boilève, Alice</creatorcontrib><creatorcontrib>Turpin, Anthony</creatorcontrib><title>Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort</title><title>Liver international</title><addtitle>Liver Int</addtitle><description>Background & Aims
Accumulating data has shown the rising incidence and poor prognosis of early‐onset gastrointestinal cancers, but few data exist on biliary tract cancers (BTC). We aimed to analyse the clinico‐pathological, molecular, therapeutic characteristics and prognosis of patients with early onset BTC (EOBTC, age ≤50 years at diagnosis), versus olders.
Methods
We analysed patients diagnosed with intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and gallbladder adenocarcinoma between 1 January 2003 and 30 June 2021. Baseline characteristics and treatment were described in each group and compared. Progression‐free survival, overall survival and disease‐free survival were estimated in each group using the Kaplan‐Meier method.
Results
Overall, 1256 patients were included, 188 (15%) with EOBTC. Patients with EOBTC demonstrated fewer comorbidities (63.5% vs. 84.5%, p < .0001), higher tumour stage (cT3–4: 50.0% vs. 32.3%, p = .0162), bilobar liver involvement (47.8% vs. 32.1%, p = .0002), and metastatic disease (67.6% vs. 57.5%, p = .0097) compared to older. Patients with EOBTC received second‐line therapy more frequently (89.5% vs. 81.0% non‐EOBTC, p = .0224). For unresectable patients with BTC, median overall survival was 17.0 vs. 16.2 months (p = .0876), and median progression‐free survival was 5.8 vs. 6.0 months (p = .8293), in EOBTC vs. older. In advanced stages, fewer actionable alterations were found in EOBTC (e.g., IDH1 mutations [7.8% vs. 16.6%]; FGFR2‐fusion [11.7% vs. 8.9%]; p = .029).
Conclusions
Patients with EOBTC have a more advanced disease at diagnosis, are treated more heavily at an advanced stage but show similar survival. A distinctive molecular profile enriched for FGRF2 fusions was found.</description><subject>Adenocarcinoma</subject><subject>Adenocarcinoma - mortality</subject><subject>Adenocarcinoma - pathology</subject><subject>Adenocarcinoma - therapy</subject><subject>Adult</subject><subject>Age of Onset</subject><subject>Aged</subject><subject>Bile Duct Neoplasms - mortality</subject><subject>Bile Duct Neoplasms - pathology</subject><subject>Bile Duct Neoplasms - therapy</subject><subject>Biliary tract</subject><subject>biliary tract cancers</subject><subject>Biliary tract diseases</subject><subject>Biliary Tract Neoplasms - mortality</subject><subject>Biliary Tract Neoplasms - pathology</subject><subject>Biliary Tract Neoplasms - therapy</subject><subject>Cancer</subject><subject>chemotherapy</subject><subject>Cholangiocarcinoma</subject><subject>Cholangiocarcinoma - mortality</subject><subject>Cholangiocarcinoma - pathology</subject><subject>Cholangiocarcinoma - therapy</subject><subject>Comorbidity</subject><subject>Diagnosis</subject><subject>early onset</subject><subject>Female</subject><subject>Fibroblast growth factor receptor 2</subject><subject>Gallbladder</subject><subject>Gallbladder cancer</subject><subject>Gallbladder Neoplasms - mortality</subject><subject>Gallbladder Neoplasms - pathology</subject><subject>Gallbladder Neoplasms - therapy</subject><subject>Humans</subject><subject>Kaplan-Meier Estimate</subject><subject>Male</subject><subject>Medical prognosis</subject><subject>Metastases</subject><subject>Middle Aged</subject><subject>molecular testing</subject><subject>Prognosis</subject><subject>Progression-Free Survival</subject><subject>Retrospective Studies</subject><subject>Survival</subject><issn>1478-3223</issn><issn>1478-3231</issn><issn>1478-3231</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><sourceid>EIF</sourceid><recordid>eNp1kctOGzEUhq2KilDKgheoLLGBRcBXxtNdEgWIFJoqarsdOfaZYjSXYHuCsuueDc_Ik-A0kEWlemPr6POnc86P0DEl5zSdi8qtzqnMGfuADqjIVJ8zTvd2b8Z76FMI94TQPJd0H_W4kkoRTg_Q061u9G-ooYm4LfHCVU77NY5em4iNbgz4gF2DQftq_fLnuW0CRLzU0aUf4Sse4AZCBIvrrorOpCJ47CH6NizBRLcCHGJn1xt5vAM8GA2GE3w9no9mc_x9Pvs2G06m2LR3rY-f0cdSVwGO3u5D9PNq_GN005_OriejwbRvuFCsry5lVuY2EwuQCyFtZkVGuQBOWKlNyQVj0hhLpeGUaiG4ynPLDNW5hVTh_BCdbr1L3z50qf2idsFAVekG2i4UnHBJMsEoSejJP-h92_kmdZcoRYnKJNsIz7aUSWMHD2Wx9K5OeywoKTYJFSmh4m9Cif3yZuwWNdgd-R5JAi62wKOrYP1_UzGd_NoqXwHW35qK</recordid><startdate>202408</startdate><enddate>202408</enddate><creator>Lebeaud, Antoine</creator><creator>Antoun, Leony</creator><creator>Paccard, Jane‐Rose</creator><creator>Edeline, Julien</creator><creator>Bourien, Hélène</creator><creator>Fares, Nadim</creator><creator>Tournigand, Christophe</creator><creator>Lecomte, Thierry</creator><creator>Tougeron, David</creator><creator>Hautefeuille, Vincent</creator><creator>Viénot, Angélique</creator><creator>Henriques, Julie</creator><creator>Williet, Nicolas</creator><creator>Bachet, Jean‐Baptiste</creator><creator>Smolenschi, Cristina</creator><creator>Hollebecque, Antoine</creator><creator>Macarulla, Teresa</creator><creator>Castet, Florian</creator><creator>Malka, David</creator><creator>Neuzillet, Cindy</creator><creator>Vernerey, Dewi</creator><creator>Boilève, Alice</creator><creator>Turpin, Anthony</creator><general>Wiley Subscription Services, Inc</general><scope>24P</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>7QO</scope><scope>7T5</scope><scope>7U9</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-2282-0101</orcidid><orcidid>https://orcid.org/0000-0002-8289-7741</orcidid><orcidid>https://orcid.org/0000-0002-5416-5383</orcidid><orcidid>https://orcid.org/0000-0001-8979-3972</orcidid></search><sort><creationdate>202408</creationdate><title>Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort</title><author>Lebeaud, Antoine ; Antoun, Leony ; Paccard, Jane‐Rose ; Edeline, Julien ; Bourien, Hélène ; Fares, Nadim ; Tournigand, Christophe ; Lecomte, Thierry ; Tougeron, David ; Hautefeuille, Vincent ; Viénot, Angélique ; Henriques, Julie ; Williet, Nicolas ; Bachet, Jean‐Baptiste ; Smolenschi, Cristina ; Hollebecque, Antoine ; Macarulla, Teresa ; Castet, Florian ; Malka, David ; Neuzillet, Cindy ; Vernerey, Dewi ; Boilève, Alice ; Turpin, Anthony</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3482-8657f9d74be5b45d7d47134e302facf34225ccd15c311a443899d2c1a9de31133</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adenocarcinoma</topic><topic>Adenocarcinoma - mortality</topic><topic>Adenocarcinoma - pathology</topic><topic>Adenocarcinoma - therapy</topic><topic>Adult</topic><topic>Age of Onset</topic><topic>Aged</topic><topic>Bile Duct Neoplasms - mortality</topic><topic>Bile Duct Neoplasms - pathology</topic><topic>Bile Duct Neoplasms - therapy</topic><topic>Biliary tract</topic><topic>biliary tract cancers</topic><topic>Biliary tract diseases</topic><topic>Biliary Tract Neoplasms - mortality</topic><topic>Biliary Tract Neoplasms - pathology</topic><topic>Biliary Tract Neoplasms - therapy</topic><topic>Cancer</topic><topic>chemotherapy</topic><topic>Cholangiocarcinoma</topic><topic>Cholangiocarcinoma - mortality</topic><topic>Cholangiocarcinoma - pathology</topic><topic>Cholangiocarcinoma - therapy</topic><topic>Comorbidity</topic><topic>Diagnosis</topic><topic>early onset</topic><topic>Female</topic><topic>Fibroblast growth factor receptor 2</topic><topic>Gallbladder</topic><topic>Gallbladder cancer</topic><topic>Gallbladder Neoplasms - mortality</topic><topic>Gallbladder Neoplasms - pathology</topic><topic>Gallbladder Neoplasms - therapy</topic><topic>Humans</topic><topic>Kaplan-Meier Estimate</topic><topic>Male</topic><topic>Medical prognosis</topic><topic>Metastases</topic><topic>Middle Aged</topic><topic>molecular testing</topic><topic>Prognosis</topic><topic>Progression-Free Survival</topic><topic>Retrospective Studies</topic><topic>Survival</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Lebeaud, Antoine</creatorcontrib><creatorcontrib>Antoun, Leony</creatorcontrib><creatorcontrib>Paccard, Jane‐Rose</creatorcontrib><creatorcontrib>Edeline, Julien</creatorcontrib><creatorcontrib>Bourien, Hélène</creatorcontrib><creatorcontrib>Fares, Nadim</creatorcontrib><creatorcontrib>Tournigand, Christophe</creatorcontrib><creatorcontrib>Lecomte, Thierry</creatorcontrib><creatorcontrib>Tougeron, David</creatorcontrib><creatorcontrib>Hautefeuille, Vincent</creatorcontrib><creatorcontrib>Viénot, Angélique</creatorcontrib><creatorcontrib>Henriques, Julie</creatorcontrib><creatorcontrib>Williet, Nicolas</creatorcontrib><creatorcontrib>Bachet, Jean‐Baptiste</creatorcontrib><creatorcontrib>Smolenschi, Cristina</creatorcontrib><creatorcontrib>Hollebecque, Antoine</creatorcontrib><creatorcontrib>Macarulla, Teresa</creatorcontrib><creatorcontrib>Castet, Florian</creatorcontrib><creatorcontrib>Malka, David</creatorcontrib><creatorcontrib>Neuzillet, Cindy</creatorcontrib><creatorcontrib>Vernerey, Dewi</creatorcontrib><creatorcontrib>Boilève, Alice</creatorcontrib><creatorcontrib>Turpin, Anthony</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Biotechnology Research Abstracts</collection><collection>Immunology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Liver international</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Lebeaud, Antoine</au><au>Antoun, Leony</au><au>Paccard, Jane‐Rose</au><au>Edeline, Julien</au><au>Bourien, Hélène</au><au>Fares, Nadim</au><au>Tournigand, Christophe</au><au>Lecomte, Thierry</au><au>Tougeron, David</au><au>Hautefeuille, Vincent</au><au>Viénot, Angélique</au><au>Henriques, Julie</au><au>Williet, Nicolas</au><au>Bachet, Jean‐Baptiste</au><au>Smolenschi, Cristina</au><au>Hollebecque, Antoine</au><au>Macarulla, Teresa</au><au>Castet, Florian</au><au>Malka, David</au><au>Neuzillet, Cindy</au><au>Vernerey, Dewi</au><au>Boilève, Alice</au><au>Turpin, Anthony</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort</atitle><jtitle>Liver international</jtitle><addtitle>Liver Int</addtitle><date>2024-08</date><risdate>2024</risdate><volume>44</volume><issue>8</issue><spage>1886</spage><epage>1899</epage><pages>1886-1899</pages><issn>1478-3223</issn><issn>1478-3231</issn><eissn>1478-3231</eissn><abstract>Background & Aims
Accumulating data has shown the rising incidence and poor prognosis of early‐onset gastrointestinal cancers, but few data exist on biliary tract cancers (BTC). We aimed to analyse the clinico‐pathological, molecular, therapeutic characteristics and prognosis of patients with early onset BTC (EOBTC, age ≤50 years at diagnosis), versus olders.
Methods
We analysed patients diagnosed with intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and gallbladder adenocarcinoma between 1 January 2003 and 30 June 2021. Baseline characteristics and treatment were described in each group and compared. Progression‐free survival, overall survival and disease‐free survival were estimated in each group using the Kaplan‐Meier method.
Results
Overall, 1256 patients were included, 188 (15%) with EOBTC. Patients with EOBTC demonstrated fewer comorbidities (63.5% vs. 84.5%, p < .0001), higher tumour stage (cT3–4: 50.0% vs. 32.3%, p = .0162), bilobar liver involvement (47.8% vs. 32.1%, p = .0002), and metastatic disease (67.6% vs. 57.5%, p = .0097) compared to older. Patients with EOBTC received second‐line therapy more frequently (89.5% vs. 81.0% non‐EOBTC, p = .0224). For unresectable patients with BTC, median overall survival was 17.0 vs. 16.2 months (p = .0876), and median progression‐free survival was 5.8 vs. 6.0 months (p = .8293), in EOBTC vs. older. In advanced stages, fewer actionable alterations were found in EOBTC (e.g., IDH1 mutations [7.8% vs. 16.6%]; FGFR2‐fusion [11.7% vs. 8.9%]; p = .029).
Conclusions
Patients with EOBTC have a more advanced disease at diagnosis, are treated more heavily at an advanced stage but show similar survival. A distinctive molecular profile enriched for FGRF2 fusions was found.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>38588031</pmid><doi>10.1111/liv.15922</doi><tpages>14</tpages><orcidid>https://orcid.org/0000-0002-2282-0101</orcidid><orcidid>https://orcid.org/0000-0002-8289-7741</orcidid><orcidid>https://orcid.org/0000-0002-5416-5383</orcidid><orcidid>https://orcid.org/0000-0001-8979-3972</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Liver international, 2024-08, Vol.44 (8), p.1886-1899 |
| issn | 1478-3223 1478-3231 1478-3231 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_3035074210 |
| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Adenocarcinoma Adenocarcinoma - mortality Adenocarcinoma - pathology Adenocarcinoma - therapy Adult Age of Onset Aged Bile Duct Neoplasms - mortality Bile Duct Neoplasms - pathology Bile Duct Neoplasms - therapy Biliary tract biliary tract cancers Biliary tract diseases Biliary Tract Neoplasms - mortality Biliary Tract Neoplasms - pathology Biliary Tract Neoplasms - therapy Cancer chemotherapy Cholangiocarcinoma Cholangiocarcinoma - mortality Cholangiocarcinoma - pathology Cholangiocarcinoma - therapy Comorbidity Diagnosis early onset Female Fibroblast growth factor receptor 2 Gallbladder Gallbladder cancer Gallbladder Neoplasms - mortality Gallbladder Neoplasms - pathology Gallbladder Neoplasms - therapy Humans Kaplan-Meier Estimate Male Medical prognosis Metastases Middle Aged molecular testing Prognosis Progression-Free Survival Retrospective Studies Survival |
| title | Management of biliary tract cancers in early‐onset patients: A nested multicenter retrospective study of the ACABI GERCOR PRONOBIL cohort |
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