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Incidence and Mortality
Cancer arising in the extrahepatic bile duct is an uncommon disease, and is curable by surgery in fewer than 10% of all cases.
Prognosis depends in part on the tumor's anatomic location, which affects its resectability. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct, a resectability rate that is clearly better than for lesions that occur in more proximal sites.
Anatomy of the extrahepatic bile duct. The extrahepatic bile duct is made up of the common hepatic duct and the common bile duct.
Bile duct cancer may occur more frequently in patients with a history of primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, or infections with the fluke, Clonorchis sinensis.
The most common symptoms caused by bile duct cancer include:
In most patients, the tumor cannot be completely removed by surgery and is incurable. Palliative resections or other palliative measures such as radiation therapy (e.g., brachytherapy or external-beam radiation therapy) or stenting procedures may maintain adequate biliary drainage and allow for improved survival. Many bile duct cancers are multifocal. Perineural invasion has a negative impact on survival.
The term, cholangiocarcinoma, is sometimes used to refer to any primary cancer of the biliary system; however, its use is often restricted to intrahepatic tumors and, therefore, it is not included in this summary. Adenocarcinomas are the most common type of extrahepatic bile duct cancers. The histologic types are listed below:[1,2]
Malignant mesenchymal tumors, although rare, include the following:
From a clinical and practical point of view, extrahepatic bile duct cancers can be considered to be localized (resectable) or unresectable. This has obvious prognostic importance.
Localized extrahepatic bile duct cancer
Patients with localized extrahepatic bile duct cancer have cancer that can be completely removed by the surgeon. These patients represent a very small minority of cases of bile duct cancer and usually are those with a lesion of the distal common bile duct where 5-year survival rate of 25% may be achieved. Extended resections of hepatic duct bifurcation tumors (Klatskin tumors, also known as hilar tumors) to include adjacent liver, either by lobectomy or removal of portions of segments 4 and 5 of the liver, may be performed. There has been no randomized trial of adjuvant therapy for patients with localized disease. Radiation therapy (external-beam radiation with or without brachytherapy), however, has been reported to improve local control.[1,2][Level of evidence: 3iiiDiii]
Klatskin tumor. A tumor that forms in the common hepatic duct, the area where the right and left hepatic duct meet.
Unresectable extrahepatic bile duct cancer
Patients with unresectable extrahepatic bile duct cancer have cancer that cannot be completely removed by the surgeon. These patients represent the majority of patients with bile duct cancer. Often the cancer invades directly into the portal vein, the adjacent liver or along the common bile duct, and to adjacent lymph nodes. Spread to distant parts of the body is uncommon but intra-abdominal metastases, particularly peritoneal metastases, do occur. At this stage, patient management is directed at palliation.
The TNM staging system should be used when staging the disease of a patient with extrahepatic bile duct cancer. Most cancers are staged following surgery and pathologic examination of the resected specimen. Evaluation of the extent of disease at laparotomy is most important for staging.
Staging depends on imaging, which often defines the limits of the tumor, and surgical exploration with pathologic examination of the resected specimen. In many cases, it may be difficult to completely resect the primary tumor.
Definitions of TNM
The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define extrahepatic bile duct cancer.[3,4] Stages defined by TNM classification apply to all primary carcinomas arising in the extrahepatic bile duct or in the cystic duct and do not apply to intrahepatic cholangiocarcinomas, sarcomas, or carcinoid tumors.[3,4]
Tables 1, 2, 3, and 4 pertain to the perihilar bile duct group.
Tables 5, 6, 7, and 8 pertain to the distal bile duct group.
In a minority of cases, proximal bile duct cancer can be completely resected. Cures are not often achieved in these patients, in contrast to patients with tumors arising in the distal bile duct, for whom a 5-year survival may be achieved in as many as 25% of patients.
Standard treatment options:
In jaundiced patients, percutaneous transhepatic catheter drainage or endoscopic placement of a stent for relief of biliary obstruction should be considered before surgery, particularly if jaundice is severe or an element of azotemia is present. An understanding of both the normal and varied vascular and ductal anatomy of the porta hepatis has increased the number of hepatic duct bifurcation tumors (Klatskin tumors) that can be resected.[1,2,3]
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized extrahepatic bile duct cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Patients with unresectable extrahepatic bile duct cancer have cancer that cannot be completely removed by the surgeon. These patients represent the majority of cases of bile duct cancer. Often a proximal bile duct cancer invades directly into the adjacent liver or into the hepatic artery or portal vein. Portal hypertension may result. Spread to distant parts of the body is uncommon, though transperitoneal and hematogenous hepatic metastases do occur with bile duct cancers of all sites. Invasion along the biliary tree and into the liver is common. Moreover, the majority of patients who undergo resection will develop recurrent disease within the hepatobiliary system or less frequently at distant sites.
Patients with unresectable, recurrent, or metastatic extrahepatic bile duct cancer should be considered for inclusion in clinical trials whenever possible. Information about ongoing clinical trials is available from the NCI Web site.
Palliative radiation therapy after biliary bypass or intubation may be beneficial, and patients may be candidates for inclusion in clinical trials that explore ways to improve the effects of radiation therapy with various radiation sensitizers, such as hyperthermia, radiosensitizer drugs, or cytotoxic chemotherapeutic agents. If a percutaneous catheter has been placed, it can be used as a conduit for placement of sources for brachytherapy.[3,4] Information about ongoing clinical trials is available from the NCI Web site.
A randomized, phase III study of up to 6 months of gemcitabine versus gemcitabine and cisplatin in 410 patients with unresectable, recurrent, or metastatic biliary tract carcinoma demonstrated an improvement in median overall survival (OS) among patients treated with combination therapy (11.7 months vs. 8.1 months; HR, 0.64; (95% confidence interval, 0.52–0.80); P < .001.[Level of evidence: 1iiA] A similar median OS benefit was demonstrated in all subgroups, including 73 patients with extrahepatic bile duct cancer and 57 patients with hilar tumors. Grade 3 and 4 toxicities occurred with similar frequency in both study arms, with the exception of increased hematologic toxicity in patients randomly assigned to gemcitabine-cisplatin and increased hepatotoxicity in patients randomly assigned to single-agent gemcitabine.
Other drugs and drug combinations await evaluation in randomized trials.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with unresectable extrahepatic bile duct cancer, recurrent extrahepatic bile duct cancer and metastatic extrahepatic bile duct cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of extrahepatic bile duct cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Extrahepatic Bile Duct Cancer Treatment are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the Web site's Contact Form. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
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National Cancer Institute: PDQ® Extrahepatic Bile Duct Cancer Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://cancer.gov/cancertopics/pdq/treatment/bileduct/HealthProfessional. Accessed <MM/DD/YYYY>.
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Last Revised: 2014-07-31
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