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Bile Duct

Disclaimer

  • This manual is not a substitute for consultation with an appropriate specialist.
  • The contents of this manual have been developed through consensus of a Provincial Tumour Group. Please note the various update dates for each section as some of the content of the manual may not be up to date.


1. Screening

There are no effective means of screening for cholangiocarcinomas at this time.

​

2. Diagnostic and Staging Workup

  • Patients usually present with jaundice, abnormal liver enzymes, pain, or dilated bile ducts on imaging
  • Patients with suspected cholangiocarcinomas should be referred to a hepatobiliary surgeon with expertise in this area
  • Initial investigations should include:
    • Liver imaging: Triphasic CT scan or contrast-enhanced MRI
    • Cholangiography either by MRCP or ERCP. (ERCP and stenting may be preferable for symptomatic patients)
    • Metastatic work-up to include CT chest
    • CEA and CA 19-9 
  • Extent of the disease should be assessed by cross-sectional imaging and cholangiography to determine proximal and distal extent of the disease.
  • A tissue diagnosis may be attempted by:
    • ERCP + brushings
    • EUS + FNA bx
    • Percutaneous biopsy of suspected mass
  • Obtaining a definitive tissue diagnosis is frequently not possible. If a lesion is potentially resectable, surgery should be considered even in the absence of a tissue diagnosis
  • If a patient has a potentially resectable lesion, preoperative drainage of the biliary tract should be considered either by ERCP or PTC. The biliary system to be drained should be determined by the type of operation being planned 
  • Laparoscopy may be considered for staging
​

3. Primary Surgical Therapy

​Distal: Whipple resection with portal lymphadenectomy
Mid-bile duct: Bile duct resection with portal lymphadenectomy. Intraoperative frozen sections should be obtained to assess margins.

Proximal: Right or left hepatectomy (including caudate lobe) with bile duct resection and portal lymphadenectomy.
Note: highly selected patients may be candidates for liver transplantation.
​

4. Pathology

  • Specimen: specify 
    • Type
    • Procedure 
  • Tumour: specify 
    • Site
    • Size
    • Histologic type
    • Histologic grade
    • Microscopic tumour extension
    • Margins
    • Lymph-Vascular invasion
    • Perineural invasion
    • pTNM (AJCC 7th edition)
      • Lymph nodes: number examined, number involved
  • Additional pathologic findings
​

5. Staging

6. Treatment Options

Localized and potentially resectable disease

  • Primary surgery as described above
  • There is no  proven role for adjuvant chemotherapy or radiotherapy for completely resected (R0) early-stage biliary cancer
  • Patients with microscopic residual disease (R1 resection) would benefit from review in multidisciplinary conference to determine suitability for adjuvant-intent chemotherapy or chemoradiation

Unresectable lesions:

  • Stenting by ERCP or PTC depending upon the location of the lesion
  • Biliary bypass is an option for unresectable disease found at time of surgery
  • Referral for palliative chemotherapy
  • Referral for radiotherapy if local symptoms (pain, bleeding, obstruction are present)

Palliative Chemotherapy for unresectable or metastatic disease

  • Patients should be referred for consideration of palliative chemotherapy, clinical trials, and/or supportive care
  • Palliative chemotherapy can extend survival in appropriately selected patients and may help improve symptoms and quality of life 
  • Currently approved chemotherapeutic agents for unresectable biliary and gall bladder cancer include: gemcitabine, cisplatin, and 5-fluorouracil (5-FU)
    • The most commonly used regimens are:
      • gemcitabine and cisplatin (GIAVPG) 
      • 5-FU and cisplatin (GIFUC)
      • single-agent 5-FU (GIAVFL)
    • The choice and sequence of chemotherapy is determined by disease-related factors, patient factors and patient preferences as assessed by the medical oncologist
  • Consider treatment on a clinical trial, if available
  • Symptom management, best supportive care, and involvement of palliative care services as indicated by patient’s clinical status

7. Follow-Up

There is no evidence that routine imaging or laboratory investigations are useful in detecting recurrences or metastases at a stage where interventions are curative. Early detection of asymptomatic metastases does not enhance survival.

Investigations should be performed based the clinical presentation of a patient who is suspected of having recurrent or metastatic disease.​

1. Screening

There are no effective means of screening for cholangiocarcinomas at this time.

​

2. Diagnostic and Staging Workup

  • Patients usually present with an isolated liver mass. Diagnostic dilemma with “metastasis of unknown primary”
  • Rule out potential sites of primary disease. This may include upper/lower GI endoscopy and/or PET scan in conjunction with tumour markers as appropriate
  • A percutaneous liver biopsy may be necessary
  • Initial investigations should include:
    • Liver imaging: Triphasic CT scan or contrast-enhanced MRI
    • Cholangiography either by MRCP or ERCP. (ERCP and stenting may be preferable for symptomatic patients)
    • Metastatic work-up/imaging to rule out potential site of primary cancer:  CT chest/abdomen/pelvis
    • CEA and CA 19-9 
    • Laparoscopy may be considered for staging
  • Obtaining a definitive tissue diagnosis is frequently not possible. If a lesion is potentially resectable, surgery should be considered even in the absence of a tissue diagnosis

3. Primary Surgical Therapy

  • Liver resection
  • If lesion < 2cm in high risk patient, consider ablation
  • Note: highly selected patients may be candidates for liver transplantation
​

4. Pathology

  • ​Specimen: specify 
    • Type
    • Procedure 
  • Tumour: specify 
    • Site
    • Size
    • Histologic type
    • Histologic grade
    • Tumour growth pattern
    • Microscopic tumour extension
    • Margins
    • Lymph-Vascular invasion
    • Perineural invasion
    • pTNM
      • Lymph nodes: number examined, number involved
  • Additional pathologic findings

5. Staging

6. Treatment Options

Localized and potentially resectable disease

  • Primary surgery as described above
  • There is no proven role for adjuvant chemotherapy or radiotherapy for completely resected early-stage biliary cancer
  • Patients with microscopic residual disease (R1 resection) would benefit from review in multidisciplinary conference to determine suitability for adjuvant-intent chemotherapy or chemoradiation

Unresectable lesions:

  • Referral for palliative chemotherapy
  • Referral for radiotherapy if local symptoms (pain, bleeding, obstruction are present)

Palliative Chemotherapy for unresectable or metastatic disease

  • Patients should be referred for consideration of palliative chemotherapy, clinical trials, and/or supportive care
  • Palliative radiotherapy may be a consideration for local symptoms (e.g. alleviation of pain bleeding or jaundice)
  • Palliative chemotherapy can extend survival in appropriately selected patients and may help improve symptoms and quality of life
  • Currently approved chemotherapeutic agents for unresectable biliary and gall bladder cancer include: gemcitabine, cisplatin, and 5-fluorouracil (5-FU)
    • The most commonly used regimens are:
      • gemcitabine and cisplatin (GIAVPG)
      • 5-FU and cisplatin (GIFUC)
      • single-agent 5-FU (GIAVFL)
    • The choice and sequence of chemotherapy is determined by disease-related factors, patient factors and patient preferences as assessed by the medical oncologist
  • Consider treatment on a clinical trial, if available
  • Symptom management, best supportive care, and involvement of palliative care services as indicated by patient’s clinical status

7. Follow-Up

There is no evidence that routine imaging or laboratory investigations are useful in detecting recurrences or metastases at a stage where interventions are curative. Early detection of asymptomatic metastases does not enhance survival.

Investigations should be performed based the clinical presentation of a patient who is suspected of having recurrent or metastatic disease.

​
SOURCE: Bile Duct ( )
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