Cancers of the gall balder and the biliary tree

Question 1

where do cholangiocarcinomas usually occur??

Answer 1

usually arises in the extrahepatic biliary system (usually at the confluence of the left and right hepatic ducts - Klatskin tumour)

Question 2

what kind of cancers are cholangiocarcinomas?

Answer 2

usually adenocarcinomas

Question 3

how do these cancers spread?

Answer 3

metastasizes to local lymph nodes, before spreading to peritoneal cavity, lung, and liver

Question 4

what are the risk factors for cholangiocarcinomas?

Answer 4

• primary sclerosing cholangitis
• ulcerative colitis
• infective (HIV hep viruses)
• toxins (chemicals in rubber and aircraft industry)
• alcohol excess
• congenital

Question 5

why do these cancers have a poor prognosis?

Answer 5

patients present late as it is generally asymptomatic until a late stage of the disease

Question 6

what are the clinical features associated with cholangiocarcinomas?

Answer 6

• post-hepatic jaundice (and pruritis)
• pale stools
• dark urine

later:

• malaise
• weight loss
• cachexia
• Courvoisier’s law*
• intrahepatic cholangiocarcinomas will present ore like hepatocellular carcinoma and will not result in symptoms of obstructive jaundice

Question 7

what investigations are performed for suspected cholangiocarcinoma?
what are the relevant findings?

Answer 7

lab tests:
LFTs –> raised ALT,GGT and bilirubin (obstructive image)
tumour markers CEA and CA19-9 may be elevated

radilogical
USS - confirms an obstructive cause 
MRCP - optimal for diagnosis 
ERCP - demonstrate site of obstruction + biopsy 
CT - staging disease (superior to MRI)

*angiography - image hepatic arteries and portal vein before op

Question 8

what is definitive management for cholangiocarcinomas?

Answer 8

complete surgical resection (depending on location)

intrahepatic / Klatskin tumours –> partial hepatectomy + reconstruction of biliary tree
distal CBD –> Whipple’s

radiotherapy may be used as adjunct or neoadjuvant treatment

Question 9

what is the palliative treatment for cholangiocarcinomas?

Answer 9

this again is the most common outcome for patients

stenting: ERCP to put in a stent relieving obstruction (often need replacement within 3-4 months)
surgery: surgical bypass procedures if stenting is not sufficient
medical: radiotherapy to extend life (potentially with chemo on top)

Question 10

what are the complications of cholangiocarcinomas?

Answer 10

• increased risk of biliary tract sepsis (obstruction)

- secondary biliary cirrhosis

Question 11

what can cause carcinoma of the gallbladder?

Answer 11

• stones –> chronic inflammation

- typically a disease of the elderly

Question 12

what condition can often present radiologically like cholangiocarcinomas and why?

Answer 12

primary sclerosing cholangitis

both involve the development of strictures which cause the obstruction and appear identical