Cholangiocarcinoma

Question 1

what is the key presentation of cholangiocarcinoma?

Answer 1

FLAWS, painless obstructive jaundice symptoms palpable gall bladder UC/PSC CA19-9

Question 2

define cholangiocarcinoma?

Answer 2

Cholangiocarcinomas are cancers arising from the bile duct epithelium. - PRIMARY ADENOCARCINOMA OF BILIARY TREE

These can be divided depending on their location in the biliary tree: intrahepatic or extrahepatic (perihilar and distal).

Question 3

what are the risk factors for cholangiocarcinoma?

Answer 3

• Ulcerative colitis + primary sclerosing cholangitis
• Choledocholithiasis + Cholecystolithiasis (stonesin bile ducts / stones in gallbladder)
• Choledochal cyst(congenital conditions involving cystic dilatations of bile ducts)
• Non-specific cirrhosis
• Alcoholic liver disease
• Caroli disease (rare genetic condition in which you get dilatation of intrahepatic bile ducts)
• HCV/ HBV/ HIV
• Parasitic infectionof biliary tract (liver fluke e.g. Chlonorchis sinesisand Opisthorchis viverrini)

Question 4

summarise the epidemiology of cholangiocarcinoma?

Answer 4

VERY RARE

More common in the developing worlddue to the increased prevalence of parasitic infections

More common in men due to increased occurrence of PSCin men

Question 5

what are the presenting symptoms of cholangiocarcinoma?

Answer 5

Obstructive jaundice symptoms

• Yellow sclera
• Pale stools
• Dark urine
• Pruritus

Abdominal pain or fullness

Systemic symptomsof malignancy: weight loss, malaise, anorexia

Question 6

what are the signs of cholangiocarcinoma on physical examination?

Answer 6

Jaundice

Palpable gallbladder which is NON-TENDER

NOTE: Courvoisier’s Law- in the presence of jaundice, a palpable gallbladder (that is non-tender) is unlikely to be due to gallstones (i.e. cancer of the pancreas or biliary tree is more likely or a distended gallbladder due to to other causes other than gallstones)

Epigastric/RUQ mass

There may be hepatomegaly

May have acute cholangitis triad: fever, jaundice, RUQ pain

Question 7

what is courvoisier’s law?

Answer 7

in the presence of jaundice, a palpable gallbladder (that is non-tender) is unlikely to be due to gallstones (i.e. cancer of the pancreas or biliary tree is more likely or a distended gallbladder due to to other causes other than gallstones)

Question 8

what are the investigations for cholangiocarcinoma and interpret the results?

Answer 8

FBC

U&Es

LFTs (high ALP + GGT + bilirubin + PTT)

Clotting screen

Tumour markers

• CEA elevated in IBD and cholangiocarcinoma (+ other tumours)
• CA19-9 is a marker of pancreatic cancer and cholangiocarcinoma
• CA-125– elevated in up to 65% of patients

Ultrasound – identifies malignant vs benign lesions

Question 9

what investigations should you consider for cholangiocarcinoma?

Answer 9

Endoscopy

ERCP will allow bile cytology and tumour biopsy

CT, MRI, Bone Scan - for staging

PTC – when tumour causes complete obstruction and ERCP is unable to assess the biliary tree proximal to the tumour