GI: Cholangiocarcinoma

Question 1

Which cancer type are cholangiocarcinomas most commonly?

Answer 1

> 90% are ductal adenocarcinomas (from aholangiocytes), the rest are squamous cell tumours

Question 2

Where can cholangiocarcinomas occur and how are they classified according to location?

Answer 2

Can occur in any part of the biliary tree:

1. intra-hepatic: small intra-hepatic bile ducts
2. peri-hilar: most common, esp. near bifurcation of right and left hepatic ducts (Klastskin tumours)
3. distal

Question 3

Where to cholangiocarcinomas typically metastasise to?

Answer 3

• local lymph nodes
• peritoneal cavity
• lung
• liver

Question 4

Name some of the risk factors for cholangiocarcinoma.

Answer 4

Often idiopathic but risk factors include:

1. Biliary tract disorders
   - primary sclerosing cholangitis (secondary to UC, 10-20% lifetime risk)
   - congenital bile duct abnormalities, e.g. choledochal cysts
2. Infective
   - liver flukes
   - chronic hepC (intrahepatic form)
   - HIV (intrahepatic form)
3. Toxins
   - rubber and aircrafft inustry
   - thorium exposure
4. Other
   - obesity
   - diabetes
   - alcohol excess

Question 5

Describe the usual presentation of cholangiocarcinoma.

Answer 5

Generally asymptomatic until late stage disease.

• post-hepatic obstruction Sx: jaundice, pruritis, pale stool and dark urine
• RUQ pain (dull ache), esp. in advanced disease
• weight loss, anorexia and malaise

+/- hepatomegaly, splenomegaly
+/- palpable gallbladder (tumours distal to cystic duct)

Question 6

What blood results would be expected in a pt with cholangiocarcinoma?

Answer 6

1. LFTs: increased bilirubin, increased GGT, increased ALP (post-hepatic jaundice features)
2. Tumour markers: CEA an CA19-9 may be raised

Question 7

What imaging would you request for a pt with suspected cholangiocarcinoma?

Answer 7

1. MRCP (gold standard for diagnosis): shows site of obstruction
2. ERCP: may be use to obtain samples for biopsy or cytological analysis
3. USS biliary tract: hilar tumours show dilation of intrahepatic biliary tree

Question 8

what is the treatment for a pt with cholangiocarcinoma?

Answer 8

Complete surgical resection is only chance of cure but <33% are resectable at diagnosis.

Palliative:

• ERCP may be used to stent the bile duct to relieve symptoms. Are prone to occlusion and may need replacing every 3mths.
• Radiotherapy may be used in some cases as adjunct or neoadjuvant or palliative therapy.