Hilar cholangiocarcinoma: expert consensus statement
Open accessAbstract
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.
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In patients without underlying chronic liver disease, overall survival after resection for hilar CCA is around 27-45% at 5 years with negative margins and 0-23% with positive margins.
“In patients without underlying chronic liver disease, overall survival rates after resection for hilar CCA are reported to be around 27-45% at 5 years with negative tumour margins (R0) and 0-23% in patients with positive margins.326”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [326]
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- Authors
- Mansour, J. C., Aloia, T. A., Crane, C. H., Heimbach, J. K., Nagino, M., & Vauthey, J.-N.
- Affiliations
- The University of Texas Southwestern Medical Center (US)
- The University of Texas MD Anderson Cancer Center (US)
- Mayo Clinic (US)
- Nagoya University (JP)
- Corresponding author
- Mansour, John C.
- ORCID
- Venue
- HPB
- Publisher
- Elsevier BV
- Published
- 2015-08
- Volume
- 17
- Issue
- 8
- Pages
- 691-699
- ISSN
- 1365-182X (ISSN-L)
- PMC
- PMC4527854
- OpenAlex
- W1741750461
- Cited by
- 414
- References
- 78
- Citations by year
- 2026: 16 · 2025: 29 · 2024: 44 · 2023: 43 · 2022: 48 · 2021: 52 · 2020: 60 · 2019: 25 · 2018: 49 · 2017: 29 · 2016: 12 · 2015: 5
- FWCI
- 31.88
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- HPB (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Cholangiocarcinoma and Gallbladder Cancer Studies
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Gallbladder and Bile Duct Disorders
- Keywords
- hilar cholangiocarcinoma, cholangiocarcinoma, multidisciplinary management, surgical resection, liver transplantation, trimodality therapy, chemoradiotherapy, preoperative biliary drainage, portal vein embolization, future liver remnant, unresectable disease, gemcitabine plus cisplatin, palliative care
- MeSH terms
- Bile Duct Neoplasms, Clinical Trials as Topic, Drainage, Hepatectomy, Humans, Neoplasm Staging, Risk Factors, Societies, Medical, United States, Stents, Incidence, Liver Transplantation, Randomized Controlled Trials as Topic, Treatment Outcome, Klatskin Tumor, Consensus, Chemoradiotherapy, Adjuvant
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