Questions About Cancer? 1-800-4-CANCER

Extrahepatic Bile Duct Cancer Treatment (PDQ®)

Health Professional Version

Stage Information for Extrahepatic Bile Duct Cancer

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; drawing shows cancer in the common hepatic duct, the area where the right and left hepatic duct meet. Also shown are the liver and gallbladder.
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.

Table 1. Primary Tumor (T)a
a Reprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.
TXPrimary tumor cannot be assessed.
T0No evidence of primary tumor.
TisCarcinoma in situ.
T1Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue.
T2aTumor invades beyond the wall of the bile duct to surrounding adipose tissue.
T2bTumor invades adjacent hepatic parenchyma.
T3Tumor invades unilateral branches of the portal vein or hepatic artery.
T4Tumor invades main portal vein or its branches bilaterally; or the common hepatic artery; or the second-order biliary radicals bilaterally; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.
Table 2. Regional Lymph Nodes (N)a
aReprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.
NXRegional lymph nodes cannot be assessed.
N0No regional lymph node metastasis.
N1Regional lymph node metastases (including nodes along the cystic duct, common bile duct, hepatic artery, and portal vein).
N2Metastases to periaortic, pericaval, superior mesenteric artery, and/or celiac artery lymph nodes.
Table 3. Distant Metastasis (M)a
aReprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.
M0No distant metastasis.
M1Distant metastasis.
Table 4. Anatomic Stage/Prognostic Groupsa
StageTNM
aReprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.
0TisN0M0
IT1N0M0
IIT2a–bN0M0
IIIAT3N0M0
IIIBT1–3N1M0
IVAT4N0–1M0
IVBAny TN2M0
Any TAny NM1

Tables 5, 6, 7, and 8 pertain to the distal bile duct group.

Table 5. Primary Tumor (T)a
aReprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.
TXPrimary tumor cannot be assessed.
T0No evidence of primary tumor.
TisCarcinoma in situ.
T1Tumor confined to the bile duct histologically.
T2Tumor invades beyond the wall of the bile duct.
T3Tumor invades the gallbladder, pancreas, duodenum, or other adjacent organs without involvement of the celiac axis or the superior mesenteric artery.
T4Tumor involves the celiac axis or the superior mesenteric artery.
Table 6. Regional Lymph Nodes (N)a
aReprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.
NXRegional lymph nodes cannot be assessed.
N0No regional lymph node metastasis.
N1Regional lymph node metastasis.
Table 7. Distant Metastasis (M)a
aReprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.
M0No distant metastasis.
M1Distant metastasis.
Table 8. Anatomic Stage/Prognostic Groupsa
StageTNM
aReprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.
0TisN0M0
IAT1N0M0
IBT2N0M0
IIAT3N0M0
IIBT1N1M0
T2N1M0
T3N1M0
IIIT4Any NM0
IVAny TAny NM1

References

  1. Kopelson G, Galdabini J, Warshaw AL, et al.: Patterns of failure after curative surgery for extra-hepatic biliary tract carcinoma: implications for adjuvant therapy. Int J Radiat Oncol Biol Phys 7 (3): 413-7, 1981. [PUBMED Abstract]
  2. Minsky BD, Wesson MF, Armstrong JG, et al.: Combined modality therapy of extrahepatic biliary system cancer. Int J Radiat Oncol Biol Phys 18 (5): 1157-63, 1990. [PUBMED Abstract]
  3. Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-22.
  4. Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.
  • Updated: July 31, 2014