Note: The American Joint Committee on Cancer has recently published a new edition of the AJCC Cancer Staging Manual, which includes revisions to the staging for this disease. The PDQ Adult Treatment Editorial Board, which is responsible for maintaining this summary, is currently reviewing the new staging to determine the changes that need to be made in the summary. In addition to updating this Stage Information section, additional changes may need to be made to other parts of this summary to ensure that it is up-to-date. The changes will be made as soon as possible.

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

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) 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]

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.

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification [3] 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]

Primary tumor (T)

• TX: Primary tumor cannot be assessed
• T0: No evidence of primary tumor
• Tis: Carcinoma in situ
• T1: Tumor confined to the bile duct histologically
• T2: Tumor invades beyond the wall of the bile duct
• T3: Tumor invades the liver, gallbladder, pancreas, and/or unilateral branches of the portal vein (right or left) or hepatic artery (right or left)
• T4: Tumor invades any of the following: main portal vein or its branches bilaterally, common hepatic artery, or other adjacent structures, such as the colon, stomach, duodenum, or abdominal wall