Cholangiocarcinoma

Question 1

Define cholangiocarcinoma.

Answer 1

Cholangiocarcinomas are cancers arising from the bile duct epithelium.

Divided depending on location: intrahepatic or extrahepatic (perihilar and distal).

Question 2

What is a Klatskin’s tumour?

Answer 2

Perihilar tumours involving the bifurcation of the ducts are also known as Klatskin’s tumours

Question 3

What cells do cholangiocarcinomas arise from? What type are most?

Answer 3

95% are adenocarcinomas - arising from epithelial cells

SCC are rare

Question 4

How common are cholangiocarcinomas?

Answer 4

66% occur between 50-70 years

Male predominance

Highest rates in South America and northern Japan

Question 5

What are the risk factors for cholangiocarcinomas?

Answer 5

• Age >50 years
• Cholangitis/ choledocholithiasis/ cholecystolithiasis
• PSC/UC
• Non-specific cirrhosis/alcoholic liver disease
• Typhoid carrier/Hep C/HIV/Hep B/ liver fluke
• Thorium dioxide exposure (radioactive contrast agent used until 1950s)

Question 6

What are the clinical features of cholangiocarcinoma?

Answer 6

Usually gradual onset obstructive pattern.

Intrahepatic

• Abdominal discomfort, malaise, nausea

Extrahepatic

• Obstructive jaundice
• Pale stool, dark urine
• Pruritus
• In advanced disease: jaundice, pruritus, weight loss, anorexia, fatigue, abdominal mass, hepatomegaly, and Courvoisier’s sign.

Question 7

When does cholangiocarcinoma usually present?

Answer 7

Cholangiocarcinoma usually presents late, with advanced disease.

Some cholangiocarcinomas are found unexpectedly as a result of an ultrasound scan or liver profile performed for a different reason.

Question 8

What is Courvoisier’s sign?

Answer 8

Painless palpable gallbladder and jaundice

Question 9

What investigations would you do for cholangiocarcinoma?

Answer 9

• LFTs - obstructive jaundice (high conjugated bilirubin, ALP and GGT, AST minimally elevated)
• PT - increased due to reduction in fat soluble vitamin absorption
• CA19-9 (85%), Ca-125 (65%), CEA - elevated

Imaging:

• US - dilated intrahepatic ducts, mass lesion
• CT/MRI - confirms diagnosis, ?localised lymphadenopathy
• ERCP is both diagnostic and therapeutic(stent placement) - shows filling defect
• PET

Question 10

What does a palpable gallbladder and painless jaundice suggest?

Answer 10

Diagnosis other than gallstones

this could be either a pancreatic ass or gallbladder stricture causing progressive ductal obstruction

Question 11

Would you do a CXR or an AXR if you suspect cholangiocarcinoma?

Answer 11

CXR first to check for metastases

US liver for lesions

Question 12

What is true of gallbladder calcification?

Answer 12

• This finding is termed porcelain gallbladder
• Associated with chronic inflammation and gallbladder carcinoma
• CT is more useful than US in the delineation of gallbladder calcification

Question 13

What is the management of cholangiocarcinoma?

Answer 13

Surgical resection - in clear margins the 5yr survival is still only up to 43%; hepatectomy or pancreaticoduodenectomy may be needed

Liver transplant

Adjuvant chemotherapy +/- radiotherapy - if positive margins after resection; improves survival

Palliation - stenting or bypass to relieve obstruction; radiofrequency ablation, transcatheter arterial embolisation.

Question 14

What are the complications of cholangiocarcinoma?

Answer 14

Cholangitis

Biliary leak - surgical complication

Biliary obstruction

Question 15

What is the prognosis with cholangiocarcinomas?

Answer 15

Node-positivity is a poor prognostic factor

The 5-year survival for surgical resection alone ranges from 20% to 43%

Recurrence with liver transplant is 51%