Cancers of Biliary Tract

Cholangiocarcinoma is a cancer originating in the rest of the biliary tract. Males are at more risk than females. It is a disease of the elderly - the 6th or 7th Decade of life. The second most common primary liver tumour. It is 3% of all Gastrointestinal cancers.

Risk Factors Of Cholangiocarcinoma

• Choledochal Cyst
• Caroli's Disease
• Primary Sclerosis Cholangitis
• Secondary Sclerosing Cholangitis
• Inflammatory Bowel Disease
• FAP
• Clonorchis Sinensis and other parasitic infections.

Genetic Predisposing Conditions

• Lynch Syndrome - eCCA and iCCA
• BAP 1 Tumour predisposition syndrome -iCCA
• Cystic Fibrosis

Types of Cholangiocarcinoma

• The most common type of Cholangiocarcinoma is Klatskin Tumour.
• The reasons for Klatskin tumour be the most common are:
  • Perihilar tumours comprise approximately 60% or 2/3rd.
• The second most common is distal cholangiocarcinoma
• The least common is iCCA - intrahepatic cholangiocarcinoma
  • It is a Subtype of intrahepatic cholangiocarcinoma
    • Mass-forming lesion – peripherally located
    • Intraductal and growing
    • Periductal infiltrating

Bismuth- Corolette Classification for Cholangiocarcinoma

• Bismuth Corolette Classification is Based on the tumour location.
• This classification mainly Concerns Perihilar Cholangiocarcinoma
• It is subdivided into 4 types depending on its location. We need to classify this as they help better manage the patients.

• Type 1 or Bismuth Corollete carcinoma
  • This type of carcinoma is more than 2 cm away from the confluence of right and left hepatic duct
•  Type 2 
  • pCCA
  • This type of carcinoma is within  2 cm of the confluence of the right and left hepatic duct.

• Type 3
  • This carcinoma is Exactly at the confluence of the right and left duct
  • It can be further type A and Type B
  • Sometimes, this type 3 cholangiocarcinoma extends into the right hepatic duct and is then subclassified as type 3A cholangiocarcinoma.
  • If the cholangiocarcinoma extends into the left hepatic duct, it is classified as type 3B cholangiocarcinoma.
• Type 4 
  • There is a tumour that invades both the right and left hepatic duct
  • This tumour is present at the confluence of both hepatic ducts.

Clinical Features of Cholagiomacarcinoma

• The Presenting complaint of cholangiocarcinoma is Obstructive Jaundice, which can be either painful or painless jaundice.
• It can be associated with abdominal pain because some fatty changes in the liver might lead to its enlargement.
• There is Weight loss seen in the patient as there is malabsorption due to an obstructive type of jaundice.
• There could be a temperature rise. 

Investigation of Choice Of Cholangiocarcinoma

• The investigation of choice is ultrasound
• Ultrasound of the liver gives insight into the detection of jaundice.

Diagnosis of Cholangiocarcinoma

• Other Investigations that can be performed
  • CT/MRI
  • MRCP detects the entire biliary tree 
• The gold standard of investigation of choice is ERCP + biopsy /FNAC– brush cytology.

Courvoisier’s Law- Cholangiocarcinoma

• On per abdominal examination, if there is
  • Distended gall bladder– originating from bile duct 
  • Jaundice.

Then Courvoisier’s Law states, "If the patient has painless jaundice and a distended, palpable gall bladder, it can not be because of gallstones.”

• If there are long-standing gallstones, the individual may have obvious acute or sub-acute cholecystitis.
• There will also be fibrotic changes, and the gallbladder will be sunken.

Management Of Cholangiocarcinoma

• The mainstay of treatment is surgical resections, which are easy when the tumour is mass-forming peripheral type.
• The limiting factor is the acceptability of the tumour
• If it is not receptible, then go for Chemotherapy or Radiotherapy
• Chemotherapy can be continued as an adjunct treatment.\
• Lobectomy is possible when its mass forms and a peripheral type
  • In perihilar regions, resectability is a challenge.
  • If possible, surgical resection should be done.
    • Invasion of portal vein
    • Meta-static with perihilar, perihilar, and porta hepatic lymph node involvement.
      • Clear the lymph node should be seen.
• The best outcome in the case of cholangiocarcinoma is when surgical resection + liver transplant +/- chemotherapy or Radiology is done.

Frequently Asked Questions

Q. What is the most common type of C6holangiocarcinoma?

Ans. Klatskin Tumour

Q. What is the gold standard of investigation of choice?

Ans. ERCP + biopsy (FNAC or brush cytology)

Q. When do you get the best outcome in case Of cholangiocarcinoma (MCQ)?

Ans. Surgical resection + liver transplant +/- chemotherapy or Radiology

Also Read: Achalasia Cardia- Clinical Features, Diagnosis And Treatment

Hope you found this blog helpful for your NEET SS Gastroenterology and Hepatobiliary preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.