Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Mar 7;30(9):1018-1042.
doi: 10.3748/wjg.v30.i9.1018.

National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

Affiliations
Review

National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

Faisal Saud Dar et al. World J Gastroenterol. .

Abstract

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute & Research Centre (PKLI & RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI & RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Keywords: Bismuth-Corlette classification; Hepatectomy; Hilar cholangiocarcinoma; Memorial Sloan Kettering Cancer Centre Staging; Portal vein embolisation; Preoperative biliary drainage; Surgical resection.

PubMed Disclaimer

Conflict of interest statement

Conflict-of-interest statement: The authors report having no conflicts of interest.

Figures

Figure 1
Figure 1
Anatomical classification of hilar cholangiocarcinoma with International Classification of Diseases-11 codes. CCA: Cholangiocarcinoma.
Figure 2
Figure 2
Bismuth-Corlette classification. Type I: Below the confluence of left and right hepatic ducts; Type II: Reaching confluence but not involving left or right hepatic ducts; Type III: occluding common hepatic duct and involving either the right (IIIa) or left (IIIb) hepatic duct; Type IV: Involving the confluence of both right and left hepatic ducts; bilateral intrahepatic segmental involvement or multicentric.

References

    1. Gatto M, Bragazzi MC, Semeraro R, Napoli C, Gentile R, Torrice A, Gaudio E, Alvaro D. Cholangiocarcinoma: update and future perspectives. Dig Liver Dis. 2010;42:253–260. - PubMed
    1. Deoliveira ML, Schulick RD, Nimura Y, Rosen C, Gores G, Neuhaus P, Clavien PA. New staging system and a registry for perihilar cholangiocarcinoma. Hepatology. 2011;53:1363–1371. - PubMed
    1. Banales JM, Cardinale V, Carpino G, Marzioni M, Andersen JB, Invernizzi P, Lind GE, Folseraas T, Forbes SJ, Fouassier L, Geier A, Calvisi DF, Mertens JC, Trauner M, Benedetti A, Maroni L, Vaquero J, Macias RI, Raggi C, Perugorria MJ, Gaudio E, Boberg KM, Marin JJ, Alvaro D. Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA) Nat Rev Gastroenterol Hepatol. 2016;13:261–280. - PubMed
    1. ICD-11 for Mortality and Morbidity Statistics. 2C18 Malignant neoplasms of perihilar bile duct. 2023. [cited 12 December 2023]. Available from: https://icd.who.int/browse11/L-m/en#/http://id.who.int/icd/entity/107123... .
    1. Blechacz BR, Gores GJ. Cholangiocarcinoma. Clin Liver Dis. 2008;12:131–150, ix. - PubMed

MeSH terms