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Cura del Colangiocarcinoma
Terapia del colangiocarcinoma
Il colangiocarcinoma è curabile?
Risposta: sì, ma purtroppo non in tutti i casi.
Il colangiocarcinoma è un tumore maligno raro che nasce dalle vie biliari. Questa malattia non ha fattori di rischio che siano stati ancora identificati e quindi non ha la possibilità di poter usufruire di programmi di prevenzione e di diagnosi precoce.
Per questo motivo il colangiocarcinoma viene spesso scoperto in fasi già avanzate della malattia:
- quando sono presenti infiltrazioni delle principali strutture del fegato,
- quando vi è un importante interessamento dei linfonodi o
- quando sono già nate delle metastasi in organi distanti, come il polmone, le ossa o il peritoneo.
In questi casi il colangiocarcinoma non è trattabile con uno scopo curativo.
Possono invece essere trattate le forme “localizzate” della malattia, quando il tumore è ancora situato solo nel fegato o nella via biliare.
Questi casi di colangiocarcinoma possono essere curati con risultati spesso soddisfacenti.
Quali sono le terapie per il colangiocarcinoma?
Risposta: La migliore terapia per curare il colangiocarcinoma è la chirurgia.
Il miglior trattamento che può eseguire oggi un paziente al quale è stato diagnosticato un colangiocarcinoma è poter eseguire un intervento chirurgico che asporti tutto il tumore e contemporaneamente rimuova i linfonodi dell’area circostante.
Gli interventi eseguibili per il colangiocarcinoma dipendono dalla sede di localizzazione del tumore nella via biliare:
- resezione epatica per i colangiocarcinomi nati dentro il fegato (c.d. “intraepatici”) e per quelli nati nel punto di incontro delle vie biliari della parte destra del fegato con le vie biliari della parte sinistra (c.d. “parailari).
- resezione della sola via biliare per i tumori nati nel coledoco, nel tratto compreso fra il fegato e la parte superiore del pancreas;
- duodenocefalopancreasectomia per i tumori nati in quella parte della via biliare che passa all’interno della testa del pancreas (c.d. “distali”).
Bisogna sottolineare che solo una percentuale di pazienti che varia dal 12 al 40% dei casi riesce effettivamente ad essere operato.
Quali sono gli esami che servono a vedere se un colangiocarcinoma è operabile?
Risposta: La TC del torace e dell’addome, eseguita con il mezzo di contrasto, è l’esame che fornisce il maggior numero di informazioni sullo stato del tumore e sulla sua possibile diffusione in altre parti del corpo, anche se la Risonanza Magnetica dell’addome è un esame qualitativamente superiore.
Altri esami che possono essere utili nello studio di un paziente con colangiocarcinoma (e che devono essere valutati a seconda dei casi singoli):
- l’ecografia endoscopica può risultare utile per le forme di colangiocarcinoma nate all’interno del pancreas;
- la colonscopia, nei casi in cui l’aspetto radiologico possa confondersi con la presenza di metastasi al fegato;
- l’ERCP (o CPRE) quando sia necessario posizionare delle protesi nella via biliare a causa della presenza di ittero;
- i marcatori tumorali CEA e CA-19-9.
La PET non è indicata fra gli esami di routine nello studio del colangiocarcinoma.
E' sempre necessario fare la biopsia se ci si trova di fronte a un colangiocarcinoma?
Risposta: No.
I pazienti che hanno o potrebbero avere l’indicazione a fare un intervento chirurgico per un colangiocarcinoma non debbono fare per forza la biopsia.
La biopsia nei casi operabili ed in cui non vi sono dubbi nella diagnosi della malattia non è indispensabile prima di essere operati.
L’esecuzione di una biopsia del fegato è invece fondamentale per chi deve eseguire la chemioterapia e in quei casi in cui vi sono delle difficoltà nel formulare una diagnosi della malattia.
Bisogna fare la chemioterapia prima di un'operazione chirurgica per un colangiocarcinoma?
Risposta: No.
Fino ad oggi non vi sono studi che supportino l’uso della chemioterapia prima di un intervento chirurgico per migliorarne i risultati.
Quindi, se un paziente ha la possibilità di poter essere operato, questa è la strada che dovrebbe essere seguita.
Nel caso di tumori che richiedono interventi tecnicamente complessi o in malattie avanzate, sono state proposte queste terapie per cercare di riportare il paziente alla possibilità di essere operato:
- Chemioembolizzazione (TACE)(20% di conversione)
- Gemcitabina
- Radioembolizzazione (SIRT)
- Radioterapia (± chemioterapia)
Che terapia deve fare un paziente che non può essere operato?
Risposta: Il paziente che non può essere operato ha l’indicazione a fare la chemioterapia sistemica.
Il trattamento di prima linea per il colangiocarcinoma che non può essere operato è la chemioterapia sistemica. In genere questi sono i trattamenti proponibili:
- Capecitabina
- Gemcitabina + oxaliplatino
- Chemioterapia intraarteriosa
- Radioterapia.
Sulla biopsia epatica che i pazienti non operabili eseguono di routine prima di iniziare la chemioterapia, dovrebbe sempre essere valutato lo stato della fusione del recettore 2 del fattore di crescita dei fibroblasti (FGFR2) che permette di iniziare una terapia mirata con il farmaco Pemigatinib.
Si può fare il trapianto di fegato se si ha un colangiocarcinoma?
Risposta: No.
Il trapianto di fegato non è fra le terapie “standard” per il colangiocarcinoma nato nel fegato (“intraepatico”) né per quello nato alla confluenza biliare (“perilare”).
In alcuni centri dove si fanno i trapianti di fegato sono in corso protocolli di ricerca per trapiantare particolari pazienti giovani con colangiocarcinoma “perilare”.
Alla fine, da chi deve farsi vedere un paziente con un probabile colangiocarcinoma?
Risposta: dal chirurgo epatobiliare o dal chirurgo epatobiliopancreatico.
Il paziente con un sospetto di colangiocarcinoma dovrebbe farsi valutare inizialmente in un centro di chirurgia del fegato, per vedere quali esami fare per completare lo studio e arrivare alla valutazione della possibilità di eseguire una eventuale operazione per asportare il tumore.
Se il paziente non ha l’indicazione a farsi operare, dovrà essere preso in carico dai medici specialisti in oncologia per la terapia più indicata.
- Ultimo aggiornamento della pagina: 07/10/2026
Linee Guida per la Diagnosi ed il Trattamento del Colangiocarcinoma
2025
Kodali S; Kulik L; D'Allessio A; Martin E D; Hakeem A R; Lewinska M; Lindsey S; Liu K; Maravic Z; Patel M S; Pinato D; Rammohan A; Rich N; Zamparelli M S; Victor D W; Vinaxia C; Brombosz E W; Villanueva A; Meyer T; Selzner N; Ghobrial R M; Rela M; Sapisochin G; Abdelrahim M; Agopian V; Balci D; Bruix J; Bekaii-Saab T S; Berenguer M; Bhangui P; Bhoori S; Chan A C Y; Chan S L; Chieh A K W; Durand F; Eghtesad B; Elsabbagh A; Halazun K J; Javle M; Hibi T; Jung D; Khalili K; Lee J M; Lewandowski R; Line P D; Llovet J M; Mazzaferro V; Mas V; Mehta N; Morement H; O'Kane G; Paradis V; Parikh N; Pillai A; Polak W; Pomposelli J; Rimassa L; Singal A; Soin A S; Tabrizian P; Toso C; Valle J; Vibert E; Vogel A; Rossi J Z; Watt K; Woods A W
The 2024 ILTS-ILCA consensus recommendations for liver transplantation for HCC and intrahepatic cholangiocarcinoma Journal Article
In: Liver Transplantation, vol. 31, no 6, pp. 815 – 831, 2025, ISSN: 15276465, (Cited by: 5).
@article{Kodali2025815,
title = {The 2024 ILTS-ILCA consensus recommendations for liver transplantation for HCC and intrahepatic cholangiocarcinoma},
author = {Sudha Kodali and Laura Kulik and Antonio D'Allessio and Eleonora De Martin and Abdul Rahman Hakeem and Monica Lewinska and Stacie Lindsey and Ken Liu and Zorana Maravic and Madhukar S. Patel and David Pinato and Ashwin Rammohan and Nicole Rich and Marco Sanduzzi Zamparelli and David W. Victor and Carmen Vinaxia and Elizabeth W. Brombosz and Augusto Villanueva and Tim Meyer and Nazia Selzner and Rafik Mark Ghobrial and Mohamed Rela and Gonzalo Sapisochin and Maen Abdelrahim and Vatche Agopian and Deniz Balci and Jordi Bruix and Tanios S. Bekaii-Saab and Marina Berenguer and Prashant Bhangui and Sherrie Bhoori and Albert Chi Yan Chan and Stephen L. Chan and Alfred Kow Wei Chieh and Francois Durand and Bijan Eghtesad and Ahmed Elsabbagh and Karim J. Halazun and Milind Javle and Taizo Hibi and Dong-Hwan Jung and Korosh Khalili and Jeong Min Lee and Robert Lewandowski and Pal Dag Line and Josep Maria Llovet and Vincenzo Mazzaferro and Valeria Mas and Neil Mehta and Helen Morement and Grainne O'Kane and Valerie Paradis and Neehar Parikh and Anjana Pillai and Wojtek Polak and James Pomposelli and Lorenza Rimassa and Amit Singal and Arvinder Singh Soin and Parissa Tabrizian and Christian Toso and Juan Valle and Eric Vibert and Arndt Vogel and Jessica Zucman Rossi and Kymberly Watt and Andrea Wilson Woods},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-86000331526&doi=10.1097%2fLVT.0000000000000589&partnerID=40&md5=10e3036c9ee0a41c6b66ff876d53ca2c},
doi = {10.1097/LVT.0000000000000589},
issn = {15276465},
year = {2025},
date = {2025-01-01},
urldate = {2025-01-01},
journal = {Liver Transplantation},
volume = {31},
number = {6},
pages = {815 – 831},
publisher = {Lippincott Williams and Wilkins},
abstract = {Liver transplantation (LT) provides the best long-term survival outcomes for patients with liver cancer. As a result, the field of transplant oncology has grown greatly over the past few decades, and many centers have expanded their criteria to allow increased access to LT for liver malignancies. Center-level guidelines and practices in transplant oncology significantly vary across the world, leading to debate regarding the best course of treatment for this patient population. An international consensus conference was convened by the International Liver Transplantation Society and the International Liver Cancer Association on February 1-2, 2024, in Valencia, Spain to establish a more universal consensus regarding LT for oncologic indications. The conference followed the Delphi process, followed by an external expert review. Consensus statements were accepted regarding patient assessment and waitlisting criteria, pretransplant treatment (including immunotherapy) and downstaging, living donor LT, post-LT patient management, and patient-and caregiver-related outcomes. The multidisciplinary participants in the consensus conference provided up-to-date recommendations regarding the selection and management of patients with liver cancer being considered for LT. Although participants deferred to center protocols in many cases, there was great interest in safely expanding access to LT for patients with larger tumor burden and biologically amenable lesions. Copyright © 2025 American Association for the Study of Liver Diseases.},
note = {Cited by: 5},
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pubstate = {published},
tppubtype = {article}
}
2024
SANITÀ S N L G D S D
Colangiocarcinoma Intraepatico e Perilare: Linee per guida per la pratica clinica Working paper
2024.
@workingpaper{nokey,
title = {Colangiocarcinoma Intraepatico e Perilare: Linee per guida per la pratica clinica},
author = {SISTEMA NAZIONALE LINEE GUIDA DELL’ISTITUTO SUPERIORE DI SANITÀ},
url = {https://www.iss.it/documents/20126/8404074/LG+100+AISF_ColangioCa_ed2024.pdf/35f5ef44-3feb-26a3-9668-f987ccac702e?t=1706188459319},
year = {2024},
date = {2024-01-25},
abstract = {Il colangiocarcinoma (CCA) è il secondo tumore epatico primitivo più comune, caratterizzato da una prognosi infausta e da resistenza ai chemioterapici. Il progressivo aumento dell'incidenza e della mortalità di CCA registrato a livello mondiale negli ultimi due decenni e la necessità di chiarire vari aspetti della gestione clinica, hanno indotto l'Associazione Italiana per lo Studio del Fegato (AISF) a commissionare la stesura di linee guida dedicate, in collaborazione con un gruppo di società scientifiche italiane dedicate all’argomento. Queste linee guida sono state formulate secondo le indicazioni dell'Istituto Superiore di Sanità e sviluppate seguendo il metodo GRADE e le relative innovazioni. Le linee guida, aggiornate al mese di Gennaio 2024, annoverano 29 Key questions. Le relative raccomandazioni affrontano gli argomenti “critici” e gli snodi decisionali più importanti nella gestione del CCA: classificazione, fattori di rischio, presentazione clinica, stadiazione, strategie diagnostiche e terapeutiche. },
keywords = {},
pubstate = {published},
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}
Roth G S; Verlingue L; Sarabi M; Blanc J; Boleslawski E; Boudjema K; Bretagne-Bignon A; Camus-Duboc M; Coriat R; Créhange G; Baere T D; Fouchardière C; Dromain C; Edeline J; Gelli M; Guiu B; Horn S; Laurent-Croise V; Lepage C; Lièvre A; Lopez A; Manfredi S; Meilleroux J; Neuzillet C; Paradis V; Prat F; Ronot M; Rosmorduc O; Cunha A S; Soubrane O; Turpin A; Louvet C; Bouché O; Malka D
In: European Journal of Cancer, vol. 202, 2024, ISSN: 09598049, (Cited by: 7).
@article{Roth2024,
title = {Biliary tract cancers: French national clinical practice guidelines for diagnosis, treatments and follow-up (TNCD, SNFGE, FFCD, UNICANCER, GERCOR, SFCD, SFED, AFEF, SFRO, SFP, SFR, ACABi, ACHBPT)},
author = {Gael S. Roth and Loic Verlingue and Matthieu Sarabi and Jean-Frédéric Blanc and Emmanuel Boleslawski and Karim Boudjema and Anne-Laure Bretagne-Bignon and Marine Camus-Duboc and Romain Coriat and Gilles Créhange and Thierry De Baere and Christelle Fouchardière and Clarisse Dromain and Julien Edeline and Maximiliano Gelli and Boris Guiu and Samy Horn and Valérie Laurent-Croise and Côme Lepage and Astrid Lièvre and Anthony Lopez and Sylvain Manfredi and Julie Meilleroux and Cindy Neuzillet and Valérie Paradis and Frédéric Prat and Maxime Ronot and Olivier Rosmorduc and Antonio Sa Cunha and Olivier Soubrane and Anthony Turpin and Christophe Louvet and Olivier Bouché and David Malka},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85188118767&doi=10.1016%2fj.ejca.2024.114000&partnerID=40&md5=e2942737317c09600207cac08af2a0ad},
doi = {10.1016/j.ejca.2024.114000},
issn = {09598049},
year = {2024},
date = {2024-01-01},
urldate = {2024-01-01},
journal = {European Journal of Cancer},
volume = {202},
publisher = {Elsevier Ltd},
abstract = {Introduction: This document is a summary of the French intergroup guidelines of the management of biliary tract cancers (BTC) (intrahepatic, perihilar and distal cholangiocarcinomas, and gallbladder carcinomas) published in September 2023, available on the website of the French Society of Gastroenterology (SNFGE) (www.tncd.org). Methods: This collaborative work was conducted under the auspices of French medical and surgical societies involved in the management of BTC. Recommendations were graded in three categories (A, B and C) according to the level of scientific evidence until August 2023. Results: BTC diagnosis and staging is mainly based on enhanced computed tomography, magnetic resonance imaging and (endoscopic) ultrasound-guided biopsy. Treatment strategy depends on BTC subtype and disease stage. Surgery followed by adjuvant capecitabine is recommended for localised disease. No neoadjuvant treatment is validated to date. Cisplatin-gemcitabine chemotherapy combined to the anti-PD-L1 inhibitor durvalumab is the first-line standard of care for advanced disease. Early systematic tumour molecular profiling is recommended to screen for actionable alterations (IDH1 mutations, FGFR2 rearrangements, HER2 amplification, BRAFV600E mutation, MSI/dMMR status, etc.) and guide subsequent lines of treatment. In the absence of actionable alterations, FOLFOX chemotherapy is the only second-line standard-of-care. No third-line chemotherapy standard is validated to date. Conclusion: These guidelines are intended to provide a personalised therapeutic strategy for daily clinical practice. Each individual BTC case should be discussed by a multidisciplinary team. © 2024 Elsevier Ltd},
note = {Cited by: 7},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
2023
Rushbrook S M; Kendall T J; Zen Y; Albazaz R; Manoharan P; Pereira S P; Sturgess R; Davidson B R; Malik H Z; Manas D; Heaton N; Prasad K R; Bridgewater J; Valle J W; Goody R; Hawkins M; Prentice W; Morement H; Walmsley M; Khan S A
British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma Journal Article
In: Gut, pp. gutjnl–2023–330029, 2023.
@article{Rushbrook2023-pd,
title = {British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma},
author = {Simon M Rushbrook and Timothy James Kendall and Yoh Zen and Raneem Albazaz and Prakash Manoharan and Stephen P Pereira and Richard Sturgess and Brian R Davidson and Hassan Z Malik and Derek Manas and Nigel Heaton and K Raj Prasad and John Bridgewater and Juan W Valle and Rebecca Goody and Maria Hawkins and Wendy Prentice and Helen Morement and Martine Walmsley and Shahid A Khan},
url = {https://gut.bmj.com/content/early/2023/09/27/gutjnl-2023-330029
https://gut.bmj.com/content/gutjnl/early/2023/09/27/gutjnl-2023-330029.full.pdf},
doi = {10.1136/gutjnl-2023-330029},
year = {2023},
date = {2023-09-01},
urldate = {2023-09-01},
journal = {Gut},
pages = {gutjnl–2023–330029},
publisher = {BMJ},
abstract = {These guidelines for the diagnosis and management of
cholangiocarcinoma (CCA) were commissioned by the British
Society of Gastroenterology liver section. The guideline writing
committee included a multidisciplinary team of experts from
various specialties involved in the management of CCA, as well
as patient/public representatives from AMMF (the
Cholangiocarcinoma Charity) and PSC Support. Quality of evidence
is presented using the Appraisal of Guidelines for Research and
Evaluation (AGREE II) format. The recommendations arising are to
be used as guidance rather than as a strict protocol-based
reference, as the management of patients with CCA is often
complex and always requires individual patient-centred
considerations.},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
cholangiocarcinoma (CCA) were commissioned by the British
Society of Gastroenterology liver section. The guideline writing
committee included a multidisciplinary team of experts from
various specialties involved in the management of CCA, as well
as patient/public representatives from AMMF (the
Cholangiocarcinoma Charity) and PSC Support. Quality of evidence
is presented using the Appraisal of Guidelines for Research and
Evaluation (AGREE II) format. The recommendations arising are to
be used as guidance rather than as a strict protocol-based
reference, as the management of patients with CCA is often
complex and always requires individual patient-centred
considerations.
easloffice@easloffice.eu E A; Liver E A
EASL-ILCA clinical practice guidelines on intrahepatic cholangiocarcinoma Journal Article
In: J. Hepatol., 2023.
@article{European_Association_for_the_Study_of_the_Liver_Electronic_address_easlofficeeaslofficeeu2023-gz,
title = {EASL-ILCA clinical practice guidelines on intrahepatic cholangiocarcinoma},
author = {European Association easloffice@easloffice.eu and European Association Liver},
url = {https://www.journal-of-hepatology.eu/article/S0168-8278(23)00185-X/fulltext
https://www.journal-of-hepatology.eu/action/showPdf?pii=S0168-8278%2823%2900185-X
},
doi = {10.1016/j.jhep.2023.03.010},
year = {2023},
date = {2023-03-01},
urldate = {2023-03-01},
journal = {J. Hepatol.},
abstract = {Intrahepatic cholangiocarcinoma (iCCA) emerges inside the liver
from bile ductules to the second-order bile ducts, represents the
second most frequent primary liver cancer besides hepatocellular
carcinoma and, unfortunately, is globally increasing in term of
incidence. The silent presentation, late diagnosis, the highly
aggressive nature and resistance to treatments lead to alarming
mortality. Early diagnosis, molecular characterization, accurate
staging and personalized multidisciplinary treatments represent
challenges for researchers and physicians. Unfortunately, these
challenges are beset by the high heterogeneity of iCCA at the
clinical, genomic, epigenetic and molecular levels that very
often preclude successful management. Nonetheless, in the last
few years, progress has been achieved in molecular
characterization, surgical management and targeted therapies. The
recent advance together with the awareness that iCCA represents a
distinct entity amongst the CCA family, led the ILCA and EASL
governing boards to commission and charge international experts
to draft dedicated guidelines to aid physicians towards an
evidence-based approach for the diagnostic, prognostic, and
therapeutic management of iCCA.},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
from bile ductules to the second-order bile ducts, represents the
second most frequent primary liver cancer besides hepatocellular
carcinoma and, unfortunately, is globally increasing in term of
incidence. The silent presentation, late diagnosis, the highly
aggressive nature and resistance to treatments lead to alarming
mortality. Early diagnosis, molecular characterization, accurate
staging and personalized multidisciplinary treatments represent
challenges for researchers and physicians. Unfortunately, these
challenges are beset by the high heterogeneity of iCCA at the
clinical, genomic, epigenetic and molecular levels that very
often preclude successful management. Nonetheless, in the last
few years, progress has been achieved in molecular
characterization, surgical management and targeted therapies. The
recent advance together with the awareness that iCCA represents a
distinct entity amongst the CCA family, led the ILCA and EASL
governing boards to commission and charge international experts
to draft dedicated guidelines to aid physicians towards an
evidence-based approach for the diagnostic, prognostic, and
therapeutic management of iCCA.
2022
Vogel A; Bridgewater J; Edeline J; Kelley R K; Klümpen H J; Malka D; Primrose J N; Rimassa L; Stenzinger A; Valle J W; Ducreux M
Biliary tract cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up Bachelor Thesis
2022, ISSN: 1569-8041.
@bachelorthesis{pmid36372281,
title = {Biliary tract cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up},
author = {A Vogel and J Bridgewater and J Edeline and R K Kelley and H J Klümpen and D Malka and J N Primrose and L Rimassa and A Stenzinger and J W Valle and M Ducreux},
doi = {10.1016/j.annonc.2022.10.506},
issn = {1569-8041},
year = {2022},
date = {2022-11-01},
urldate = {2022-11-01},
journal = {Ann Oncol},
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Bowlus C L; Arrivé L; Bergquist A; Deneau M; Forman L; Ilyas S I; Lunsford K E; Martinez M; Sapisochin G; Shroff R; Tabibian J H; Assis D N
AASLD Practice Guidance on Primary Sclerosing Cholangitis and Cholangiocarcinoma Journal Article
In: Hepatology, 2022, ISSN: 1527-3350.
@article{pmid36083140,
title = {AASLD Practice Guidance on Primary Sclerosing Cholangitis and Cholangiocarcinoma},
author = {Christopher L Bowlus and Lionel Arrivé and Annika Bergquist and Mark Deneau and Lisa Forman and Sumera I Ilyas and Keri E Lunsford and Mercedes Martinez and Gonzalo Sapisochin and Rachna Shroff and James H Tabibian and David N Assis},
doi = {10.1002/hep.32771},
issn = {1527-3350},
year = {2022},
date = {2022-09-01},
urldate = {2022-09-01},
journal = {Hepatology},
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}
per lo Studio del Fegato (AISF) A I; International Hepato-PancreatoBiliaryAssociation I; di Oncologia Medica (AIOM) A I; Group A T C C U C; di Chirurgia (SIC) S I; di Gastroenterologia ed Endoscopia digestiva (SIGE) S I; di Radiologia Medica ed Interventistica (SIRM) S I; d’Organo (SITO) S I T
Colangiocarcinoma intraepatico e perilare. Linee Guida per la pratica clinica. Working paper
Sistema Nazionale Linee Guida (SNLG) dell’Istituto Superiore di Sanità., 2022.
@workingpaper{nokey,
title = {Colangiocarcinoma intraepatico e perilare. Linee Guida per la pratica clinica.},
author = {Associazione Italiana per lo Studio del Fegato (AISF) and International Hepato-PancreatoBiliaryAssociation,
(IT-IHPBA) and Associazione Italiana di Oncologia Medica (AIOM) and AMMF -The Cholangiocarcinoma
Charity (UK) CholangiocarcinomaWorking Group and Società Italiana di Chirurgia (SIC) and Società Italiana di
Gastroenterologia ed Endoscopia digestiva (SIGE) and Società Italiana di Radiologia Medica ed Interventistica
(SIRM) and Società Italiana Trapianti d’Organo (SITO)},
url = {https://snlg.iss.it/wp-content/uploads/2022/02/LG-100-AISF_ColangioCa.pdf},
year = {2022},
date = {2022-02-20},
urldate = {2022-02-20},
howpublished = {Sistema Nazionale Linee Guida (SNLG) dell’Istituto Superiore di Sanità.},
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Kubo S; Shinkawa H; Asaoka Y; Ioka T; Igaki H; Izumi N; Itoi T; Unno M; Ohtsuka M; Okusaka T; Kadoya M; Kudo M; Kumada T; Kokudo N; Sakamoto M; Sakamoto Y; Sakurai H; Takayama T; Nakashima O; Nagata Y; Hatano E; Harada K; Murakami T; Yamamoto M
Liver Cancer Study Group of Japan Clinical Practice Guidelines for Intrahepatic Cholangiocarcinoma Journal Article
In: Liver Cancer, vol. 11, no 4, pp. 290 – 314, 2022, ISSN: 22351795, (Cited by: 0; All Open Access, Gold Open Access, Green Open Access).
@article{Kubo2022290,
title = {Liver Cancer Study Group of Japan Clinical Practice Guidelines for Intrahepatic Cholangiocarcinoma},
author = {Shoji Kubo and Hiroji Shinkawa and Yoshinari Asaoka and Tatsuya Ioka and Hiroshi Igaki and Namiki Izumi and Takao Itoi and Michiaki Unno and Masayuki Ohtsuka and Takuji Okusaka and Masumi Kadoya and Masatoshi Kudo and Takashi Kumada and Norihiro Kokudo and Michiie Sakamoto and Yoshihiro Sakamoto and Hideyuki Sakurai and Tadatoshi Takayama and Osamu Nakashima and Yasushi Nagata and Etsuro Hatano and Kenichi Harada and Takamichi Murakami and Masakazu Yamamoto},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85134222582&doi=10.1159%2f000522403&partnerID=40&md5=85a60f3437ed401dcf4b7154c820b351},
doi = {10.1159/000522403},
issn = {22351795},
year = {2022},
date = {2022-01-01},
urldate = {2022-01-01},
journal = {Liver Cancer},
volume = {11},
number = {4},
pages = {290 – 314},
publisher = {S. Karger AG},
abstract = {This paper presents the first version of clinical practice guidelines for intrahepatic cholangiocarcinoma (ICC) established by the Liver Cancer Study Group of Japan. These guidelines consist of 1 treatment algorithm, 5 background statements, 16 clinical questions, and 1 clinical topic, including etiology, staging, pathology, diagnosis, and treatments. Globally, a high incidence of ICC has been reported in East and Southeast Asian countries, and the incidence has been gradually increasing in Japan and also in Western countries. Reported risk factors for ICC include cirrhosis, hepatitis B/C, alcohol consumption, diabetes, obesity, smoking, nonalcoholic steatohepatitis, and liver fluke infestation, as well as biliary diseases, such as primary sclerosing cholangitis, hepatolithiasis, congenital cholangiectasis, and Caroli disease. Chemical risk factors include thorium-232, 1,2-dichloropropane, and dichloromethane. CA19-9 and CEA are recommended as tumor markers for early detection and diagnostic of ICC. Abdominal ultrasonography, CT, and MRI are effective imaging modalities for diagnosing ICC. If bile duct invasion is suspected, imaging modalities for examining the bile ducts may be useful. In unresectable cases, tumor biopsy should be considered when deemed necessary for the differential diagnosis and drug therapy selection. The mainstay of treatment for patients with Child-Pugh class A or B liver function is surgical resection and drug therapy. If the patient has no regional lymph node metastasis (LNM) and has a single tumor, resection is the treatment of choice. If both regional LNM and multiple tumors are present, drug therapy is the first treatment of choice. If the patient has either regional LNM or multiple tumors, resection or drug therapy is selected, depending on the extent of metastasis or the number of tumors. If distant metastasis is present, drug therapy is the treatment of choice. Percutaneous ablation therapy may be considered for patients who are ineligible for surgical resection or drug therapy due to decreased hepatic functional reserve or comorbidities. For unresectable ICC without extrahepatic metastasis, stereotactic radiotherapy (tumor size ≤5 cm) or particle radiotherapy (no size restriction) may be considered. ICC is generally not indicated for liver transplantation, and palliative care is recommended for patients with Child-Pugh class C liver function. © 2022 The Author(s).},
note = {Cited by: 0; All Open Access, Gold Open Access, Green Open Access},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
2021
Nagino M; Hirano S; Yoshitomi H; Aoki T; Uesaka K; Unno M; Ebata T; Konishi M; Sano K; Shimada K; Shimizu H; Higuchi R; Wakai T; Isayama H; Okusaka T; Tsuyuguchi T; Hirooka Y; Furuse J; Maguchi H; Suzuki K; Yamazaki H; Kijima H; Yanagisawa A; Yoshida M; Yokoyama Y; Mizuno T; Endo I
Clinical practice guidelines for the management of biliary tract cancers 2019: The 3rd English edition Journal Article
In: Journal of Hepato-Biliary-Pancreatic Sciences, vol. 28, no 1, pp. 26 – 54, 2021, ISSN: 18686974, (Cited by: 41).
@article{Nagino202126,
title = {Clinical practice guidelines for the management of biliary tract cancers 2019: The 3rd English edition},
author = {Masato Nagino and Satoshi Hirano and Hideyuki Yoshitomi and Taku Aoki and Katsuhiko Uesaka and Michiaki Unno and Tomoki Ebata and Masaru Konishi and Keiji Sano and Kazuaki Shimada and Hiroaki Shimizu and Ryota Higuchi and Toshifumi Wakai and Hiroyuki Isayama and Takuji Okusaka and Toshio Tsuyuguchi and Yoshiki Hirooka and Junji Furuse and Hiroyuki Maguchi and Kojiro Suzuki and Hideya Yamazaki and Hiroshi Kijima and Akio Yanagisawa and Masahiro Yoshida and Yukihiro Yokoyama and Takashi Mizuno and Itaru Endo},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85098000027&doi=10.1002%2fjhbp.870&partnerID=40&md5=e2f525f8a8276af922fc928beb153725},
doi = {10.1002/jhbp.870},
issn = {18686974},
year = {2021},
date = {2021-01-01},
urldate = {2021-01-01},
journal = {Journal of Hepato-Biliary-Pancreatic Sciences},
volume = {28},
number = {1},
pages = {26 – 54},
publisher = {Blackwell Publishing Asia},
abstract = {Background: The Japanese Society of Hepato-Biliary-Pancreatic Surgery launched the clinical practice guidelines for the management of biliary tract cancers (cholangiocarcinoma, gallbladder cancer, and ampullary cancer) in 2007, then published the 2nd version in 2014. Methods: In this 3rd version, clinical questions (CQs) were proposed on six topics. The recommendation, grade for recommendation, and statement for each CQ were discussed and finalized by an evidence-based approach. Recommendations were graded as Grade 1 (strong) or Grade 2 (weak) according to the concepts of the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. Results: The 31 CQs covered the six topics: (a) prophylactic treatment, (b) diagnosis, (c) biliary drainage, (d) surgical treatment, (e) chemotherapy, and (f) radiation therapy. In the 31 CQs, 14 recommendations were rated strong and 14 recommendations weak. The remaining three CQs had no recommendation. Each CQ includes a statement of how the recommendations were graded. Conclusions: This latest guideline provides recommendations for important clinical aspects based on evidence. Future collaboration with the cancer registry will be key for assessing the guidelines and establishing new evidence. © 2020 Japanese Society of Hepato-Biliary-Pancreatic Surgery},
note = {Cited by: 41},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
2020
Alvaro D; Hassan C; Cardinale V; Carpino G; Fabris L; Gringeri E; Granata V; Mutignani M; Morement H; Giuliante F; Guglielmi A; Ridola L; Tonini G; Marzioni M; Grazi G; Guido M; Giulio E D; Pantano F; Venere R; Bragazzi M C; Biancanello F; Faccioli J; Giannetti A; Cintolo M; Giunta M D; Gambato M; Lasagni A; Izzo F; Avallone A; Banales J; Rossi M; Catalano C; Laghi A; D'amati G; Mancino M G
Italian Clinical Practice Guidelines on Cholangiocarcinoma – Part II: Treatment Journal Article
In: Digestive and Liver Disease, vol. 52, no 12, pp. 1430 – 1442, 2020, ISSN: 15908658, (Cited by: 20).
@article{Alvaro20201430,
title = {Italian Clinical Practice Guidelines on Cholangiocarcinoma – Part II: Treatment},
author = {Domenico Alvaro and Cesare Hassan and Vincenzo Cardinale and Guido Carpino and Luca Fabris and Enrico Gringeri and Vincenza Granata and Massimiliano Mutignani and Helen Morement and Felice Giuliante and Alfredo Guglielmi and Lorenzo Ridola and Giuseppe Tonini and Marco Marzioni and Gianluca Grazi and Maria Guido and Emilio Di Giulio and Francesco Pantano and Rosanna Venere and Maria Consiglia Bragazzi and Francesca Biancanello and Jessica Faccioli and Aurora Giannetti and Marcello Cintolo and Michela Di Giunta and Martina Gambato and Alberto Lasagni and Francesco Izzo and Antonio Avallone and Jesus Banales and Massimo Rossi and Carlo Catalano and Andrea Laghi and Giulia D'amati and Maria Grazia Mancino},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85097004048&doi=10.1016%2fj.dld.2020.08.030&partnerID=40&md5=0d57247083149aa3927829d7d27ec2de},
doi = {10.1016/j.dld.2020.08.030},
issn = {15908658},
year = {2020},
date = {2020-01-01},
urldate = {2020-01-01},
journal = {Digestive and Liver Disease},
volume = {52},
number = {12},
pages = {1430 – 1442},
publisher = {Elsevier B.V.},
abstract = {Currently, the only curative treatment for cholangiocarcinoma (CCA) is surgical resection, though this treatment is possible in less than 40% of patients. However, recent improvements in preoperative management have led to a higher number of patients who are candidates for this procedure. For unresectable patients, progress is ongoing in terms of locoregional and chemoradiation treatments and target therapies, especially in the definition of patient selection criteria. This is the second part of the Italian CCA guidelines, dealing with CCA treatment, that have been formulated in accordance with Italian National Institute of Health indications and developed according to the GRADE method and related advancements. © 2020 Editrice Gastroenterologica Italiana S.r.l.},
note = {Cited by: 20},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
Alvaro D; Hassan C; Cardinale V; Carpino G; Fabris L; Gringeri E; Granata V; Mutignani M; Morement H; Giuliante F; Guglielmi A; Ridola L; Marzioni M; Grazi G; Guido M; Giulio E D; Tonini G; Pantano F; Venere R; Bragazzi M C; Biancanello F; Faccioli J; Giannetti A; Cintolo M; Giunta M D; Gambato M; Lasagni A; Izzo F; Avallone A; Banales J; Rossi M; Catalano C; Laghi A; D'amati G; Mancino M G
Italian Clinical Practice Guidelines on Cholangiocarcinoma – Part I: Classification, diagnosis and staging Journal Article
In: Digestive and Liver Disease, vol. 52, no 11, pp. 1282 – 1293, 2020, ISSN: 15908658, (Cited by: 23).
@article{Alvaro20201282,
title = {Italian Clinical Practice Guidelines on Cholangiocarcinoma – Part I: Classification, diagnosis and staging},
author = {Domenico Alvaro and Cesare Hassan and Vincenzo Cardinale and Guido Carpino and Luca Fabris and Enrico Gringeri and Vincenza Granata and Massimiliano Mutignani and Helen Morement and Felice Giuliante and Alfredo Guglielmi and Lorenzo Ridola and Marco Marzioni and Gianluca Grazi and Maria Guido and Emilio Di Giulio and Giuseppe Tonini and Francesco Pantano and Rosanna Venere and Maria Consiglia Bragazzi and Francesca Biancanello and Jessica Faccioli and Aurora Giannetti and Marcello Cintolo and Michela Di Giunta and Martina Gambato and Alberto Lasagni and Francesco Izzo and Antonio Avallone and Jesus Banales and Massimo Rossi and Carlo Catalano and Andrea Laghi and Giulia D'amati and Maria Grazia Mancino},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85090489680&doi=10.1016%2fj.dld.2020.06.045&partnerID=40&md5=506a895d20e629c0f967b85df789dea7},
doi = {10.1016/j.dld.2020.06.045},
issn = {15908658},
year = {2020},
date = {2020-01-01},
urldate = {2020-01-01},
journal = {Digestive and Liver Disease},
volume = {52},
number = {11},
pages = {1282 – 1293},
publisher = {Elsevier B.V.},
abstract = {Cholangiocarcinoma (CCA) is the second most common primary liver cancer, characterized by a poor prognosis and resistance to chemotherapeutics. The progressive increase in CCA incidence and mortality registered worldwide in the last two decades and the need to clarify various aspects of clinical management have prompted the Italian Association for the Study of the Liver (AISF) to commission the drafting of dedicated guidelines in collaboration with a group of Italian scientific societies. These guidelines have been formulated in accordance with the Italian National Institute of Health indications and developed by following the GRADE method and related advancements. © 2020 Editrice Gastroenterologica Italiana S.r.l.},
note = {Cited by: 23},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
2016
Valle J W; Borbath I; Khan S A; Huguet F; Gruenberger T; Arnold D; Committee O E G
Biliary cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow-up Journal Article
In: Annals of Oncology, vol. 27, pp. v28 – v37, 2016, ISSN: 09237534, (Cited by: 329; All Open Access, Bronze Open Access).
@article{Valle2016v28,
title = {Biliary cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow-up},
author = {J. W. Valle and I. Borbath and S. A. Khan and F. Huguet and T. Gruenberger and D. Arnold and On ESMO Guidelines Committee},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-84995766824&doi=10.1093%2fannonc%2fmdw324&partnerID=40&md5=13cce31dda4f3895d07e2dea0d1c53d4},
doi = {10.1093/annonc/mdw324},
issn = {09237534},
year = {2016},
date = {2016-01-01},
urldate = {2016-01-01},
journal = {Annals of Oncology},
volume = {27},
pages = {v28 – v37},
publisher = {Oxford University Press},
note = {Cited by: 329; All Open Access, Bronze Open Access},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
2015
Weber S M; Ribero D; O'Reilly E M; Kokudo N; Miyazaki M; Pawlik T M
Intrahepatic Cholangiocarcinoma: Expert consensus statement Journal Article
In: HPB, vol. 17, no 8, pp. 669 – 680, 2015, ISSN: 1365182X, (Cited by: 239; All Open Access, Bronze Open Access, Green Open Access).
@article{Weber2015669,
title = {Intrahepatic Cholangiocarcinoma: Expert consensus statement},
author = {Sharon M. Weber and Dario Ribero and Eileen M. O'Reilly and Norihiro Kokudo and Masaru Miyazaki and Timothy M. Pawlik},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-84936990315&doi=10.1111%2fhpb.12441&partnerID=40&md5=44758468378be4a8cbc6a6af005d471c},
doi = {10.1111/hpb.12441},
issn = {1365182X},
year = {2015},
date = {2015-01-01},
urldate = {2015-01-01},
journal = {HPB},
volume = {17},
number = {8},
pages = {669 – 680},
publisher = {Blackwell Publishing Ltd},
abstract = {An American Hepato-Pancreato-Biliary Association (AHPBA)-sponsored consensus meeting of expert panellists met on 15 January 2014 to review current evidence on the management of intrahepatic cholangiocarcinoma (ICC) in order to establish practice guidelines and to agree on consensus statements. The treatment of ICC requires a coordinated, multidisciplinary approach to optimize survival. Biopsy is not necessary if the surgeon suspects ICC and is planning curative resection, although biopsy should be obtained before systemic or locoregional therapies are initiated. Assessment of resectability is best accomplished using cross-sectional imaging [computed tomography (CT) or magnetic resonance imaging (MRI)], but the role of positron emission tomography (PET) is unclear. Resectability in ICC is defined by the ability to completely remove the disease while leaving an adequate liver remnant. Extrahepatic disease, multiple bilobar or multicentric tumours, and lymph node metastases beyond the primary echelon are contraindications to resection. Regional lymphadenectomy should be considered a standard part of surgical therapy. In patients with high-risk features, the routine use of diagnostic laparoscopy is recommended. The preoperative diagnosis of combined hepatocellular carcinoma and cholangiocarcinoma (cHCC-CC) by imaging studies is extremely difficult. Surgical resection remains the mainstay of treatment, but survival is worse than in HCC alone. There are no adequately powered, randomized Phase III trials that can provide definitive recommendations for adjuvant therapy for ICC. Patients with high-risk features (lymphovascular invasion, multicentricity or satellitosis, large tumours) should be encouraged to enrol in clinical trials and to consider adjuvant therapy. Cisplatin plus gemcitabine represents the standard-of-care, front-line systemic therapy for metastatic ICC. Genomic analyses of biliary cancers support the development of targeted therapeutic interventions. © 2015 International Hepato-Pancreato-Biliary Association.},
note = {Cited by: 239; All Open Access, Bronze Open Access, Green Open Access},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
Mansour J C; Aloia T A; Crane C H; Heimbach J K; Nagino M; Vauthey J
Hilar Cholangiocarcinoma: Expert consensus statement Journal Article
In: HPB, vol. 17, no 8, pp. 691 – 699, 2015, ISSN: 1365182X, (Cited by: 219; All Open Access, Bronze Open Access, Green Open Access).
@article{Mansour2015691,
title = {Hilar Cholangiocarcinoma: Expert consensus statement},
author = {John C. Mansour and Thomas A. Aloia and Christopher H. Crane and Julie K. Heimbach and Masato Nagino and Jean-Nicolas Vauthey},
url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-84936991861&doi=10.1111%2fhpb.12450&partnerID=40&md5=c91ee89909b1b8597130265eaa6ed597},
doi = {10.1111/hpb.12450},
issn = {1365182X},
year = {2015},
date = {2015-01-01},
urldate = {2015-01-01},
journal = {HPB},
volume = {17},
number = {8},
pages = {691 – 699},
publisher = {Blackwell Publishing Ltd},
abstract = {An American Hepato-Pancreato-Biliary Association (AHPBA)-sponsored consensus meeting of expert panellists met on 15 January 2014 to review current evidence on the management of hilar cholangiocarcinoma in order to establish practice guidelines and to agree consensus statements. It was established that the treatment of patients with hilar cholangiocarcinoma requires a coordinated, multidisciplinary approach to optimize the chances for both durable survival and effective palliation. An adequate diagnostic and staging work-up includes high-quality cross-sectional imaging; however, pathologic confirmation is not required prior to resection or initiation of a liver transplant trimodal treatment protocol. The ideal treatment for suitable patients with resectable hilar malignancy is resection of the intra- and extrahepatic bile ducts, as well as resection of the involved ipsilateral liver. Preoperative biliary drainage is best achieved with percutaneous transhepatic approaches and may be indicated for patients with cholangitis, malnutrition or hepatic insufficiency. Portal vein embolization is a safe and effective strategy for increasing the future liver remnant (FLR) and is particularly useful for patients with an FLR of <30%. Selected patients with unresectable hilar cholangiocarcinoma should be evaluated for a standard trimodal protocol incorporating external beam and endoluminal radiation therapy, systemic chemotherapy and liver transplantation. Post-resection chemoradiation should be offered to patients who show high-risk features on surgical pathology. Chemoradiation is also recommended for patients with locally advanced, unresectable hilar cancers. For patients with locally recurrent or metastatic hilar cholangiocarcinoma, first-line chemotherapy with gemcitabine and cisplatin is recommended based on multiple Phase II trials and a large randomized controlled trial including a heterogeneous population of patients with biliary cancers. © 2015 International Hepato-Pancreato-Biliary Association.},
note = {Cited by: 219; All Open Access, Bronze Open Access, Green Open Access},
keywords = {},
pubstate = {published},
tppubtype = {article}
}

