Guidelines for Cholangiocarcinoma Show Disconnect Between Goals, Implementation


Recommendations for the management of bile duct cancer benefit authorities, patients, and physicians, but investigators say more research is needed.

Guidelines for the management cholangiocarcinoma, or bile duct cancer, can benefit authorities, patients, and physicians, but there is a disconnect between expected goals and implementation, according to the results of a study published in Intractable & Rare Diseases Research.

Investigators conducted literary searches using Cochrane, Embase, and PubMed, as well as bibliography searches and manual searches of association websites, to build consensus and determine guidelines for the management of cholangiocarcinoma.

The search was limited to studies in either Chinese or English and ranged from 2012 to July 31, 2022. Investigators used keywords, such as “biliary tract cancer,” “cholangiocarcinoma,” “consensus,” “guideline,” and “hepatobiliary cancers.”

Investigators examined the guidelines extensively and compared and contrasted the recommendations. They identified the main differences and gaps in the guidelines.

They included 14 guidelines and consensus for cholangiocarcinoma, worldwide, including 3 from the United States, 4 from Asia, and 7 from Europe.

Investigators found that managing cholangiocarcinoma remains challenging, but there is uniformity among the various recommendations.

However, in the past 10 years, there have not been major improvements in surgical treatments.

Also, systemic therapy for cholangiocarcinoma is a key clinical issue, with no promising breakthroughs.

Investigators found that further research into locally advanced and metastatic cholangiocarcinoma is needed, and they think that target therapy could be beneficial.

Additionally, though immunotherapy for cholangiocarcinoma has shown positive results, there must be more clinical evidence before recommendations can be made, according to investigators, who noted that the purpose of the guidelines is not to replace physicians’ expertise but to provide them with the most up-to-date options for patients.

There are still crucial issues for optimal treatment options for care that must be addressed, investigators said.

Further, governments should develop and implement domestic recommendations for advance standard management of cholangiocarcinoma, investigators said.

Recommendations should be appropriate to specific patients, evidence-based, resource-constrained, and subject to systematic evolutions, they said.

Investigators also analyzed the screening and diagnosis of cholangiocarcinoma. They found that the guidelines contain distinctive diagnostic algorithms, but there is no accurate imaging examination for a comprehensive evaluation.

All the guidelines included histological, imaging, and serological diagnosis.

The most used staging systems for cholangiocarcinoma are those from the American Joint Committee on Cancer (AJCC) and the Union for International Cancer Control.

Four of 11 guidelines were revised after they were published, while the other 5 may not have been revised, because of the AJCC system was published in 2016 and made effective in 2018, investigators said.

The most significant alteration was made in the N stage for cholangiocarcinoma. In the 8th edition, the lymph node staging of individuals with cholangiocarcinoma was altered with the N1- and N2-stage categories based on positive lymph nodes.

In 1 study the investigators analyzed, results showed that approximately 40% of patients had their stage changed when the 7th edition was changed to the 8th edition.


Zhang X, Cai Y, Xiong X, et al. Comparison of current guidelines and consensus on the management of patients with cholangiocarcinoma: 2022 update. Intractable Rare Dis Res. 2022;11(4):161-172. doi:10.5582/irdr.2022.01109

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