Cholangiocarcinoma is an uncommon tumour that can arise anywhere in the biliary tree and accounts for 1.5% of all cancers. It is an adenocarcinoma.
It can arise in:
- Intrahepatic bile ducts (20-25%)
- Confluence of left and right hepatic ducts at the liver hilum (50-60%)
- Distal common bile duct (20%)
Chronic biliary inflammation:
- Primary sclerosing cholangitis
- Caroli disease (congenital cystic dilation of intrahepatic ducts)
- Choledochal cyst
- Chronic liver infection
- Obstructive jaundice
- Upper abdominal pain
- Weight loss
- LFT: raised bilirubin and ALP
- FBC: anaemia, leucocytosis
- Raised serum tumour marker CA19-9
- ERCP – can also be used to biopsy
- CT scan: staging
If the tumour has not spread, surgical excision of the extrahepatic biliary tree with or without liver resection can be performed. This improves 5-year survival from 5% to 20%.
However, most patients have tumours that have spread at the time of presentation. Only palliative therapies to relieve biliary obstruction can be offered in such cases, which include stenting across the stricture using ERCP or bypass procedures (e.g. anastomosing jejunum and hepatic duct)
Cholangiocarcinoma has a poor prognosis. Resection offer the only potential cure, although most present with metastases.