Cholangiocarcinoma

Question 1

What is cholangiocarcinoma?

Answer 1

Cancer of the biliary system.

It can occur at any site but most commonly in extrahepatic biliary system

Question 2

What is the most common site for bile duct cancers?

Answer 2

Bifurcation of the right and left hepatic ducts (Klatskin tumours)

Question 3

Nature of Klatskin tumours

Answer 3

Slow-growing tumours that invade locally

They metastasise to local lymph nodes before spreading distally.

They then spread to peritoneal cavity, lung and liver.

Question 4

Epidemiology

Answer 4

Two thirds of cases in patients >65 years

Higher incidence in SE asia due to chronic endemic parasitic infections from liver flukes.

Question 5

Cholangiocarcinoma histology.

Answer 5

95% are adenocarcinomas from cholangiocytes within the biliary tree.

Remaining are SCC, or even rarer like sarcomas, lymphomas and small cell cancers

Question 6

Risk factors

Answer 6

PSC

UC

Infective like liver flukes, HIV, hepatitis viruses

Toxins like chemical in rubber and aircraft industry

Congenital (Caroli’s disease, choledochal cyst)

Alcohol excess

Dm

Question 7

Clinical features

Answer 7

Generally asymptomatic until late stage

Post-hepatic jaundice + pruritus

Pale stools and dark urine

Less commonly…

RUQ pain, early satiety, weight loss, anorexia and malaise

Question 8

Examination findings

Answer 8

Jaundice and cachexia

Courvoisier’s law can be applied

Question 9

Explain Courvoisier’s law.

Answer 9

If there is presence of jaundice and an enlarged or palpable gallbladder

-> malignancy of the biliary tree or pancrease should be strongly suspected since the cause is unlikely to be gallstones.

Question 10

Dx

Answer 10

Post-hepatic jaundice causes

Obstructive choledocholithiasis

Bile duct strictures

Choledochal cysts

External compression from extra-biliary tumours like…
Benign biliary tumours
Pancreatic tumours
PBC
PSC

Question 11

Lab tests

Answer 11

Biochemistry for bilirubin, ALP and gamma-GT

Tumour markers - CEA and CA19-9 may also be elevated

Question 12

Imaging

Answer 12

USS may be used initially to confirm an obstructive cause.

MRCP is the optimal imaging for diagnosis

ERCP can demonstrate site of obstruction and can also be used to obtain smaples for cytology and histology.

Question 13

How is staging assessed?

Answer 13

CT imaging

It is better than MRI because it is better at locating distant metastases

Angiography may be used in pre-op planning to image the hepatic arteries or portal vein

Question 14

Definitive management

Answer 14

Complete surgical resection (majority of patients have inoperable disease at time of presentation however)

Question 15

Management of intrahepatic Klatskin tumours

Answer 15

Partial hepatectomy and reconstruction of the biliary tree

Question 16

Management of distal common duct tumours

Answer 16

Pancreaticduodenectomy (Whipple’s procedure)

Question 17

What other mangement can be used?

Answer 17

Radiotherapy is sometimes used neo or adjunct.

Post-op chemo is not readily used.

Question 18

Palliative management

Answer 18

Stenting by ERCP to relieve obstructive symptoms

Surgery by bypass to relieve obstruction

Palliative radiotherapy can be used to prolong survival.

Chemo with cisplatin and gemcitabine can also slow it down.

Question 19

Complications

Answer 19

Biliary tract sepsis due to biliary obstruction

Secondary biliary cirrhosis

Average survival is 12-18 months from diagnosis due to late presentation

5 year survival rate 10-40%